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About
What is Reactome ?
News
Team
Scientific Advisory Board
Editorial Calendar
Statistics
Our Logo
License Agreement
Privacy Notice
Disclaimer
Content
Table of Contents
DOIs
Data Schema
Reactome Research Spotlight
ORCID Integration Project
COVID-19 Disease Pathways
Docs
Userguide
Pathway Browser
How do I search ?
Details Panel
Analysis Tools
Analysis Data
Analysis Gene Expression
Species Comparison
Tissue Distribution
Diseases
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H2O [lysosomal lumen]
Stable Identifier
R-ALL-1605715
Type
Chemical Compound [SimpleEntity]
Compartment
lysosomal lumen
Synonyms
water
Icon
Locations in the PathwayBrowser
for Species:
Homo sapiens
Bos taurus
Caenorhabditis elegans
Canis familiaris
Danio rerio
Dictyostelium discoideum
Drosophila melanogaster
Gallus gallus
Mus musculus
Rattus norvegicus
Saccharomyces cerevisiae
Schizosaccharomyces pombe
Sus scrofa
Xenopus tropicalis
Expand all
Metabolism (Bos taurus)
Metabolism of carbohydrates (Bos taurus)
Glycogen metabolism (Bos taurus)
Glycogen breakdown (glycogenolysis) (Bos taurus)
GAA hydrolyzes lysosomal glycogen (Bos taurus)
H2O [lysosomal lumen]
Glycosaminoglycan metabolism (Bos taurus)
Chondroitin sulfate/dermatan sulfate metabolism (Bos taurus)
CS/DS degradation (Bos taurus)
ARSB hydrolyses C4S/C6S chains (Bos taurus)
H2O [lysosomal lumen]
ARSB hydrolyses DS (Bos taurus)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from DS (Bos taurus)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Bos taurus)
H2O [lysosomal lumen]
HYAL1 hydrolyses Chondroitin chains (Bos taurus)
H2O [lysosomal lumen]
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Bos taurus)
H2O [lysosomal lumen]
IDUA) hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Bos taurus)
H2O [lysosomal lumen]
Heparan sulfate/heparin (HS-GAG) metabolism (Bos taurus)
HS-GAG degradation (Bos taurus)
GLB1 hydrolyses linker chain(2) (Bos taurus)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses CS/HS precursor (Bos taurus)
H2O [lysosomal lumen]
Heparanase (HPSE) cleaves heparan sulfate from its proteoglycan (lysosome) (Bos taurus)
H2O [lysosomal lumen]
IDS hydrolyses Heparan sulfate chain(5) (Bos taurus)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(1) (Bos taurus)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(6) (Bos taurus)
H2O [lysosomal lumen]
NAGLU hydrolyses Heparan sulfate chain(4) (Bos taurus)
H2O [lysosomal lumen]
NAGLU hydrolyses heparan chain(2) (Bos taurus)
H2O [lysosomal lumen]
SGSH hydrolyses Heparan sulfate chain(2) (Bos taurus)
H2O [lysosomal lumen]
SGSH hydrolyses Heparan sulfate chain(7) (Bos taurus)
H2O [lysosomal lumen]
Hyaluronan metabolism (Bos taurus)
Hyaluronan uptake and degradation (Bos taurus)
GUSB tetramer hydrolyses (HA)2 (Bos taurus)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Bos taurus)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from small HA fragments (Bos taurus)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from small HA fragments (Bos taurus)
H2O [lysosomal lumen]
HYAL1 hydrolyses (HA)50 (Bos taurus)
H2O [lysosomal lumen]
Keratan sulfate/keratin metabolism (Bos taurus)
Keratan sulfate degradation (Bos taurus)
GALNS oligomer hydrolyses sulfate from Gal6S in keratan sulfate (Bos taurus)
H2O [lysosomal lumen]
GLB1 hydrolyses a glycosaminoglycan (Bos taurus)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from keratan sulfate (Bos taurus)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Bos taurus)
H2O [lysosomal lumen]
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Bos taurus)
H2O [lysosomal lumen]
Lysosomal oligosaccharide catabolism (Bos taurus)
MAN2B1 hydrolyses GlcNAc (Man)5 to GlcNAc (Man)3 (Bos taurus)
H2O [lysosomal lumen]
MAN2B2 hydrolyses GlcNAc (Man)3 to GlcNAc:Man (Bos taurus)
H2O [lysosomal lumen]
Metabolism of lipids (Bos taurus)
Fatty acid metabolism (Bos taurus)
Fatty acyl-CoA biosynthesis (Bos taurus)
PPT1 hydrolyses palmitoylated proteins (Bos taurus)
H2O [lysosomal lumen]
PPT2 hydrolyses PALMCoA to PALM (Bos taurus)
H2O [lysosomal lumen]
Sphingolipid metabolism (Bos taurus)
Glycosphingolipid metabolism (Bos taurus)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Bos taurus)
H2O [lysosomal lumen]
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Bos taurus)
H2O [lysosomal lumen]
Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside (Bos taurus)
H2O [lysosomal lumen]
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Bos taurus)
H2O [lysosomal lumen]
Beta-galactosidase hydrolyses GM1 to GM2 (Bos taurus)
H2O [lysosomal lumen]
Both hexosaminidase A and B can cleave GalNAc from globoside (Bos taurus)
H2O [lysosomal lumen]
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Bos taurus)
H2O [lysosomal lumen]
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Bos taurus)
H2O [lysosomal lumen]
Hexosaminidase A cleaves GalNAc from GM2 to form GM3 (Bos taurus)
H2O [lysosomal lumen]
Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen) (Bos taurus)
H2O [lysosomal lumen]
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Bos taurus)
H2O [lysosomal lumen]
Metabolism of proteins (Bos taurus)
Post-translational protein modification (Bos taurus)
Asparagine N-linked glycosylation (Bos taurus)
Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein (Bos taurus)
Synthesis of substrates in N-glycan biosythesis (Bos taurus)
Sialic acid metabolism (Bos taurus)
NEU1 hydrolyses Neu5Ac from glycoconjugates (Bos taurus)
H2O [lysosomal lumen]
NEU4 hydrolyses Neu5Ac from glycoconjugates (Bos taurus)
H2O [lysosomal lumen]
Transport to the Golgi and subsequent modification (Bos taurus)
N-glycan antennae elongation in the medial/trans-Golgi (Bos taurus)
Reactions specific to the complex N-glycan synthesis pathway (Bos taurus)
FUCA1 hydrolyses NGP:1,6-GlcNAc (Bos taurus)
H2O [lysosomal lumen]
Neuronal System (Bos taurus)
Transmission across Chemical Synapses (Bos taurus)
Neurotransmitter release cycle (Bos taurus)
NAAA hydrolyses NAEs to FAs and ethanolamine (Bos taurus)
H2O [lysosomal lumen]
Transport of small molecules (Bos taurus)
Plasma lipoprotein assembly, remodeling, and clearance (Bos taurus)
Plasma lipoprotein clearance (Bos taurus)
LDL clearance (Bos taurus)
LIPA hydrolyses sterol esters to sterols and fatty acids (Bos taurus)
H2O [lysosomal lumen]
Metabolism (Caenorhabditis elegans)
Metabolism of carbohydrates (Caenorhabditis elegans)
Glycosaminoglycan metabolism (Caenorhabditis elegans)
Chondroitin sulfate/dermatan sulfate metabolism (Caenorhabditis elegans)
CS/DS degradation (Caenorhabditis elegans)
HEXA cleaves the terminal GalNAc from DS (Caenorhabditis elegans)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Caenorhabditis elegans)
H2O [lysosomal lumen]
HYAL1 hydrolyses Chondroitin chains (Caenorhabditis elegans)
H2O [lysosomal lumen]
Heparan sulfate/heparin (HS-GAG) metabolism (Caenorhabditis elegans)
HS-GAG degradation (Caenorhabditis elegans)
GLB1 hydrolyses linker chain(2) (Caenorhabditis elegans)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses CS/HS precursor (Caenorhabditis elegans)
H2O [lysosomal lumen]
NAGLU hydrolyses Heparan sulfate chain(4) (Caenorhabditis elegans)
H2O [lysosomal lumen]
NAGLU hydrolyses heparan chain(2) (Caenorhabditis elegans)
H2O [lysosomal lumen]
Hyaluronan metabolism (Caenorhabditis elegans)
Hyaluronan uptake and degradation (Caenorhabditis elegans)
GUSB tetramer hydrolyses (HA)2 (Caenorhabditis elegans)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Caenorhabditis elegans)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from small HA fragments (Caenorhabditis elegans)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from small HA fragments (Caenorhabditis elegans)
H2O [lysosomal lumen]
HYAL1 hydrolyses (HA)50 (Caenorhabditis elegans)
H2O [lysosomal lumen]
Keratan sulfate/keratin metabolism (Caenorhabditis elegans)
Keratan sulfate degradation (Caenorhabditis elegans)
GLB1 hydrolyses a glycosaminoglycan (Caenorhabditis elegans)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from keratan sulfate (Caenorhabditis elegans)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Caenorhabditis elegans)
H2O [lysosomal lumen]
Lysosomal oligosaccharide catabolism (Caenorhabditis elegans)
MAN2B1 hydrolyses GlcNAc (Man)5 to GlcNAc (Man)3 (Caenorhabditis elegans)
H2O [lysosomal lumen]
MAN2B2 hydrolyses GlcNAc (Man)3 to GlcNAc:Man (Caenorhabditis elegans)
H2O [lysosomal lumen]
Metabolism of lipids (Caenorhabditis elegans)
Fatty acid metabolism (Caenorhabditis elegans)
Fatty acyl-CoA biosynthesis (Caenorhabditis elegans)
PPT1 hydrolyses palmitoylated proteins (Caenorhabditis elegans)
H2O [lysosomal lumen]
Sphingolipid metabolism (Caenorhabditis elegans)
Glycosphingolipid metabolism (Caenorhabditis elegans)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Caenorhabditis elegans)
H2O [lysosomal lumen]
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Caenorhabditis elegans)
H2O [lysosomal lumen]
Beta-galactosidase hydrolyses GM1 to GM2 (Caenorhabditis elegans)
H2O [lysosomal lumen]
Both hexosaminidase A and B can cleave GalNAc from globoside (Caenorhabditis elegans)
H2O [lysosomal lumen]
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Caenorhabditis elegans)
H2O [lysosomal lumen]
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Caenorhabditis elegans)
H2O [lysosomal lumen]
Hexosaminidase A cleaves GalNAc from GM2 to form GM3 (Caenorhabditis elegans)
H2O [lysosomal lumen]
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Caenorhabditis elegans)
H2O [lysosomal lumen]
Metabolism of proteins (Caenorhabditis elegans)
Post-translational protein modification (Caenorhabditis elegans)
Asparagine N-linked glycosylation (Caenorhabditis elegans)
Transport to the Golgi and subsequent modification (Caenorhabditis elegans)
N-glycan antennae elongation in the medial/trans-Golgi (Caenorhabditis elegans)
Reactions specific to the complex N-glycan synthesis pathway (Caenorhabditis elegans)
FUCA1 hydrolyses NGP:1,6-GlcNAc (Caenorhabditis elegans)
H2O [lysosomal lumen]
Neuronal System (Caenorhabditis elegans)
Transmission across Chemical Synapses (Caenorhabditis elegans)
Neurotransmitter release cycle (Caenorhabditis elegans)
NAAA hydrolyses NAEs to FAs and ethanolamine (Caenorhabditis elegans)
H2O [lysosomal lumen]
Metabolism (Canis familiaris)
Metabolism of carbohydrates (Canis familiaris)
Glycosaminoglycan metabolism (Canis familiaris)
Chondroitin sulfate/dermatan sulfate metabolism (Canis familiaris)
CS/DS degradation (Canis familiaris)
ARSB hydrolyses C4S/C6S chains (Canis familiaris)
H2O [lysosomal lumen]
ARSB hydrolyses DS (Canis familiaris)
H2O [lysosomal lumen]
HYAL1 hydrolyses Chondroitin chains (Canis familiaris)
H2O [lysosomal lumen]
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Canis familiaris)
H2O [lysosomal lumen]
IDUA) hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Canis familiaris)
H2O [lysosomal lumen]
Heparan sulfate/heparin (HS-GAG) metabolism (Canis familiaris)
HS-GAG degradation (Canis familiaris)
GLB1 hydrolyses linker chain(2) (Canis familiaris)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses CS/HS precursor (Canis familiaris)
H2O [lysosomal lumen]
Heparanase (HPSE) cleaves heparan sulfate from its proteoglycan (lysosome) (Canis familiaris)
H2O [lysosomal lumen]
IDS hydrolyses Heparan sulfate chain(5) (Canis familiaris)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(1) (Canis familiaris)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(6) (Canis familiaris)
H2O [lysosomal lumen]
NAGLU hydrolyses Heparan sulfate chain(4) (Canis familiaris)
H2O [lysosomal lumen]
NAGLU hydrolyses heparan chain(2) (Canis familiaris)
H2O [lysosomal lumen]
SGSH hydrolyses Heparan sulfate chain(2) (Canis familiaris)
H2O [lysosomal lumen]
SGSH hydrolyses Heparan sulfate chain(7) (Canis familiaris)
H2O [lysosomal lumen]
Hyaluronan metabolism (Canis familiaris)
Hyaluronan uptake and degradation (Canis familiaris)
GUSB tetramer hydrolyses (HA)2 (Canis familiaris)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Canis familiaris)
H2O [lysosomal lumen]
HYAL1 hydrolyses (HA)50 (Canis familiaris)
H2O [lysosomal lumen]
Keratan sulfate/keratin metabolism (Canis familiaris)
Keratan sulfate degradation (Canis familiaris)
GALNS oligomer hydrolyses sulfate from Gal6S in keratan sulfate (Canis familiaris)
H2O [lysosomal lumen]
GLB1 hydrolyses a glycosaminoglycan (Canis familiaris)
H2O [lysosomal lumen]
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Canis familiaris)
H2O [lysosomal lumen]
Lysosomal oligosaccharide catabolism (Canis familiaris)
MAN2B1 hydrolyses GlcNAc (Man)5 to GlcNAc (Man)3 (Canis familiaris)
H2O [lysosomal lumen]
MAN2B2 hydrolyses GlcNAc (Man)3 to GlcNAc:Man (Canis familiaris)
H2O [lysosomal lumen]
Metabolism of lipids (Canis familiaris)
Fatty acid metabolism (Canis familiaris)
Fatty acyl-CoA biosynthesis (Canis familiaris)
PPT1 hydrolyses palmitoylated proteins (Canis familiaris)
H2O [lysosomal lumen]
PPT2 hydrolyses PALMCoA to PALM (Canis familiaris)
H2O [lysosomal lumen]
Sphingolipid metabolism (Canis familiaris)
Glycosphingolipid metabolism (Canis familiaris)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Canis familiaris)
H2O [lysosomal lumen]
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Canis familiaris)
H2O [lysosomal lumen]
Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside (Canis familiaris)
H2O [lysosomal lumen]
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Canis familiaris)
H2O [lysosomal lumen]
Beta-galactosidase hydrolyses GM1 to GM2 (Canis familiaris)
H2O [lysosomal lumen]
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Canis familiaris)
H2O [lysosomal lumen]
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Canis familiaris)
H2O [lysosomal lumen]
Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen) (Canis familiaris)
H2O [lysosomal lumen]
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Canis familiaris)
H2O [lysosomal lumen]
Metabolism of proteins (Canis familiaris)
Post-translational protein modification (Canis familiaris)
Asparagine N-linked glycosylation (Canis familiaris)
Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein (Canis familiaris)
Synthesis of substrates in N-glycan biosythesis (Canis familiaris)
Sialic acid metabolism (Canis familiaris)
NEU1 hydrolyses Neu5Ac from glycoconjugates (Canis familiaris)
H2O [lysosomal lumen]
Transport to the Golgi and subsequent modification (Canis familiaris)
N-glycan antennae elongation in the medial/trans-Golgi (Canis familiaris)
Reactions specific to the complex N-glycan synthesis pathway (Canis familiaris)
FUCA1 hydrolyses NGP:1,6-GlcNAc (Canis familiaris)
H2O [lysosomal lumen]
Transport of small molecules (Canis familiaris)
Plasma lipoprotein assembly, remodeling, and clearance (Canis familiaris)
Plasma lipoprotein clearance (Canis familiaris)
LDL clearance (Canis familiaris)
LIPA hydrolyses sterol esters to sterols and fatty acids (Canis familiaris)
H2O [lysosomal lumen]
Metabolism (Danio rerio)
Metabolism of carbohydrates (Danio rerio)
Glycosaminoglycan metabolism (Danio rerio)
Chondroitin sulfate/dermatan sulfate metabolism (Danio rerio)
CS/DS degradation (Danio rerio)
ARSB hydrolyses C4S/C6S chains (Danio rerio)
H2O [lysosomal lumen]
ARSB hydrolyses DS (Danio rerio)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from DS (Danio rerio)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Danio rerio)
H2O [lysosomal lumen]
HYAL1 hydrolyses Chondroitin chains (Danio rerio)
H2O [lysosomal lumen]
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Danio rerio)
H2O [lysosomal lumen]
IDUA) hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Danio rerio)
H2O [lysosomal lumen]
Heparan sulfate/heparin (HS-GAG) metabolism (Danio rerio)
HS-GAG degradation (Danio rerio)
GLB1 hydrolyses linker chain(2) (Danio rerio)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses CS/HS precursor (Danio rerio)
H2O [lysosomal lumen]
Heparanase (HPSE) cleaves heparan sulfate from its proteoglycan (lysosome) (Danio rerio)
H2O [lysosomal lumen]
IDS hydrolyses Heparan sulfate chain(5) (Danio rerio)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(1) (Danio rerio)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(6) (Danio rerio)
H2O [lysosomal lumen]
NAGLU hydrolyses Heparan sulfate chain(4) (Danio rerio)
H2O [lysosomal lumen]
NAGLU hydrolyses heparan chain(2) (Danio rerio)
H2O [lysosomal lumen]
SGSH hydrolyses Heparan sulfate chain(2) (Danio rerio)
H2O [lysosomal lumen]
SGSH hydrolyses Heparan sulfate chain(7) (Danio rerio)
H2O [lysosomal lumen]
Hyaluronan metabolism (Danio rerio)
Hyaluronan uptake and degradation (Danio rerio)
GUSB tetramer hydrolyses (HA)2 (Danio rerio)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Danio rerio)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from small HA fragments (Danio rerio)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from small HA fragments (Danio rerio)
H2O [lysosomal lumen]
HYAL1 hydrolyses (HA)50 (Danio rerio)
H2O [lysosomal lumen]
Keratan sulfate/keratin metabolism (Danio rerio)
Keratan sulfate degradation (Danio rerio)
GALNS oligomer hydrolyses sulfate from Gal6S in keratan sulfate (Danio rerio)
H2O [lysosomal lumen]
GLB1 hydrolyses a glycosaminoglycan (Danio rerio)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from keratan sulfate (Danio rerio)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Danio rerio)
H2O [lysosomal lumen]
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Danio rerio)
H2O [lysosomal lumen]
Lysosomal oligosaccharide catabolism (Danio rerio)
MAN2B1 hydrolyses GlcNAc (Man)5 to GlcNAc (Man)3 (Danio rerio)
H2O [lysosomal lumen]
MAN2B2 hydrolyses GlcNAc (Man)3 to GlcNAc:Man (Danio rerio)
H2O [lysosomal lumen]
Metabolism of lipids (Danio rerio)
Fatty acid metabolism (Danio rerio)
Fatty acyl-CoA biosynthesis (Danio rerio)
PPT1 hydrolyses palmitoylated proteins (Danio rerio)
H2O [lysosomal lumen]
PPT2 hydrolyses PALMCoA to PALM (Danio rerio)
H2O [lysosomal lumen]
Sphingolipid metabolism (Danio rerio)
Glycosphingolipid metabolism (Danio rerio)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Danio rerio)
H2O [lysosomal lumen]
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Danio rerio)
H2O [lysosomal lumen]
Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside (Danio rerio)
H2O [lysosomal lumen]
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Danio rerio)
H2O [lysosomal lumen]
Beta-galactosidase hydrolyses GM1 to GM2 (Danio rerio)
H2O [lysosomal lumen]
Both hexosaminidase A and B can cleave GalNAc from globoside (Danio rerio)
H2O [lysosomal lumen]
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Danio rerio)
H2O [lysosomal lumen]
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Danio rerio)
H2O [lysosomal lumen]
Hexosaminidase A cleaves GalNAc from GM2 to form GM3 (Danio rerio)
H2O [lysosomal lumen]
Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen) (Danio rerio)
H2O [lysosomal lumen]
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Danio rerio)
H2O [lysosomal lumen]
Metabolism of proteins (Danio rerio)
Post-translational protein modification (Danio rerio)
Asparagine N-linked glycosylation (Danio rerio)
Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein (Danio rerio)
Synthesis of substrates in N-glycan biosythesis (Danio rerio)
Sialic acid metabolism (Danio rerio)
NEU1 hydrolyses Neu5Ac from glycoconjugates (Danio rerio)
H2O [lysosomal lumen]
NEU4 hydrolyses Neu5Ac from glycoconjugates (Danio rerio)
H2O [lysosomal lumen]
Transport to the Golgi and subsequent modification (Danio rerio)
N-glycan antennae elongation in the medial/trans-Golgi (Danio rerio)
Reactions specific to the complex N-glycan synthesis pathway (Danio rerio)
FUCA1 hydrolyses NGP:1,6-GlcNAc (Danio rerio)
H2O [lysosomal lumen]
Transport of small molecules (Danio rerio)
Plasma lipoprotein assembly, remodeling, and clearance (Danio rerio)
Plasma lipoprotein clearance (Danio rerio)
LDL clearance (Danio rerio)
LIPA hydrolyses sterol esters to sterols and fatty acids (Danio rerio)
H2O [lysosomal lumen]
Metabolism (Dictyostelium discoideum)
Metabolism of carbohydrates (Dictyostelium discoideum)
Glycosaminoglycan metabolism (Dictyostelium discoideum)
Chondroitin sulfate/dermatan sulfate metabolism (Dictyostelium discoideum)
CS/DS degradation (Dictyostelium discoideum)
HEXA cleaves the terminal GalNAc from DS (Dictyostelium discoideum)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Dictyostelium discoideum)
H2O [lysosomal lumen]
IDUA) hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Dictyostelium discoideum)
H2O [lysosomal lumen]
Heparan sulfate/heparin (HS-GAG) metabolism (Dictyostelium discoideum)
HS-GAG degradation (Dictyostelium discoideum)
GLB1 hydrolyses linker chain(2) (Dictyostelium discoideum)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(1) (Dictyostelium discoideum)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(6) (Dictyostelium discoideum)
H2O [lysosomal lumen]
NAGLU hydrolyses Heparan sulfate chain(4) (Dictyostelium discoideum)
H2O [lysosomal lumen]
NAGLU hydrolyses heparan chain(2) (Dictyostelium discoideum)
H2O [lysosomal lumen]
Hyaluronan metabolism (Dictyostelium discoideum)
Hyaluronan uptake and degradation (Dictyostelium discoideum)
HEXA cleaves the terminal GalNAc from small HA fragments (Dictyostelium discoideum)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from small HA fragments (Dictyostelium discoideum)
H2O [lysosomal lumen]
Keratan sulfate/keratin metabolism (Dictyostelium discoideum)
Keratan sulfate degradation (Dictyostelium discoideum)
GLB1 hydrolyses a glycosaminoglycan (Dictyostelium discoideum)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from keratan sulfate (Dictyostelium discoideum)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Dictyostelium discoideum)
H2O [lysosomal lumen]
Lysosomal oligosaccharide catabolism (Dictyostelium discoideum)
MAN2B1 hydrolyses GlcNAc (Man)5 to GlcNAc (Man)3 (Dictyostelium discoideum)
H2O [lysosomal lumen]
MAN2B2 hydrolyses GlcNAc (Man)3 to GlcNAc:Man (Dictyostelium discoideum)
H2O [lysosomal lumen]
Metabolism of lipids (Dictyostelium discoideum)
Fatty acid metabolism (Dictyostelium discoideum)
Fatty acyl-CoA biosynthesis (Dictyostelium discoideum)
PPT1 hydrolyses palmitoylated proteins (Dictyostelium discoideum)
H2O [lysosomal lumen]
PPT2 hydrolyses PALMCoA to PALM (Dictyostelium discoideum)
H2O [lysosomal lumen]
Sphingolipid metabolism (Dictyostelium discoideum)
Glycosphingolipid metabolism (Dictyostelium discoideum)
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Dictyostelium discoideum)
H2O [lysosomal lumen]
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Dictyostelium discoideum)
H2O [lysosomal lumen]
Beta-galactosidase hydrolyses GM1 to GM2 (Dictyostelium discoideum)
H2O [lysosomal lumen]
Both hexosaminidase A and B can cleave GalNAc from globoside (Dictyostelium discoideum)
H2O [lysosomal lumen]
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Dictyostelium discoideum)
H2O [lysosomal lumen]
Metabolism of proteins (Dictyostelium discoideum)
Post-translational protein modification (Dictyostelium discoideum)
Asparagine N-linked glycosylation (Dictyostelium discoideum)
Transport to the Golgi and subsequent modification (Dictyostelium discoideum)
N-glycan antennae elongation in the medial/trans-Golgi (Dictyostelium discoideum)
Reactions specific to the complex N-glycan synthesis pathway (Dictyostelium discoideum)
FUCA1 hydrolyses NGP:1,6-GlcNAc (Dictyostelium discoideum)
H2O [lysosomal lumen]
Metabolism (Drosophila melanogaster)
Metabolism of carbohydrates (Drosophila melanogaster)
Glycosaminoglycan metabolism (Drosophila melanogaster)
Chondroitin sulfate/dermatan sulfate metabolism (Drosophila melanogaster)
CS/DS degradation (Drosophila melanogaster)
HEXA cleaves the terminal GalNAc from DS (Drosophila melanogaster)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Drosophila melanogaster)
H2O [lysosomal lumen]
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Drosophila melanogaster)
H2O [lysosomal lumen]
IDUA) hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Drosophila melanogaster)
H2O [lysosomal lumen]
Heparan sulfate/heparin (HS-GAG) metabolism (Drosophila melanogaster)
HS-GAG degradation (Drosophila melanogaster)
GLB1 hydrolyses linker chain(2) (Drosophila melanogaster)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses CS/HS precursor (Drosophila melanogaster)
H2O [lysosomal lumen]
Heparanase (HPSE) cleaves heparan sulfate from its proteoglycan (lysosome) (Drosophila melanogaster)
H2O [lysosomal lumen]
IDS hydrolyses Heparan sulfate chain(5) (Drosophila melanogaster)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(1) (Drosophila melanogaster)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(6) (Drosophila melanogaster)
H2O [lysosomal lumen]
NAGLU hydrolyses Heparan sulfate chain(4) (Drosophila melanogaster)
H2O [lysosomal lumen]
NAGLU hydrolyses heparan chain(2) (Drosophila melanogaster)
H2O [lysosomal lumen]
SGSH hydrolyses Heparan sulfate chain(2) (Drosophila melanogaster)
H2O [lysosomal lumen]
SGSH hydrolyses Heparan sulfate chain(7) (Drosophila melanogaster)
H2O [lysosomal lumen]
Hyaluronan metabolism (Drosophila melanogaster)
Hyaluronan uptake and degradation (Drosophila melanogaster)
GUSB tetramer hydrolyses (HA)2 (Drosophila melanogaster)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Drosophila melanogaster)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from small HA fragments (Drosophila melanogaster)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from small HA fragments (Drosophila melanogaster)
H2O [lysosomal lumen]
Keratan sulfate/keratin metabolism (Drosophila melanogaster)
Keratan sulfate degradation (Drosophila melanogaster)
GLB1 hydrolyses a glycosaminoglycan (Drosophila melanogaster)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from keratan sulfate (Drosophila melanogaster)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Drosophila melanogaster)
H2O [lysosomal lumen]
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Drosophila melanogaster)
H2O [lysosomal lumen]
Lysosomal oligosaccharide catabolism (Drosophila melanogaster)
MAN2B1 hydrolyses GlcNAc (Man)5 to GlcNAc (Man)3 (Drosophila melanogaster)
H2O [lysosomal lumen]
MAN2B2 hydrolyses GlcNAc (Man)3 to GlcNAc:Man (Drosophila melanogaster)
H2O [lysosomal lumen]
Metabolism of lipids (Drosophila melanogaster)
Fatty acid metabolism (Drosophila melanogaster)
Fatty acyl-CoA biosynthesis (Drosophila melanogaster)
PPT1 hydrolyses palmitoylated proteins (Drosophila melanogaster)
H2O [lysosomal lumen]
PPT2 hydrolyses PALMCoA to PALM (Drosophila melanogaster)
H2O [lysosomal lumen]
Sphingolipid metabolism (Drosophila melanogaster)
Glycosphingolipid metabolism (Drosophila melanogaster)
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Drosophila melanogaster)
H2O [lysosomal lumen]
Beta-galactosidase hydrolyses GM1 to GM2 (Drosophila melanogaster)
H2O [lysosomal lumen]
Both hexosaminidase A and B can cleave GalNAc from globoside (Drosophila melanogaster)
H2O [lysosomal lumen]
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Drosophila melanogaster)
H2O [lysosomal lumen]
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Drosophila melanogaster)
H2O [lysosomal lumen]
Metabolism of proteins (Drosophila melanogaster)
Post-translational protein modification (Drosophila melanogaster)
Asparagine N-linked glycosylation (Drosophila melanogaster)
Transport to the Golgi and subsequent modification (Drosophila melanogaster)
N-glycan antennae elongation in the medial/trans-Golgi (Drosophila melanogaster)
Reactions specific to the complex N-glycan synthesis pathway (Drosophila melanogaster)
FUCA1 hydrolyses NGP:1,6-GlcNAc (Drosophila melanogaster)
H2O [lysosomal lumen]
Metabolism (Gallus gallus)
Metabolism of carbohydrates (Gallus gallus)
Glycosaminoglycan metabolism (Gallus gallus)
Chondroitin sulfate/dermatan sulfate metabolism (Gallus gallus)
CS/DS degradation (Gallus gallus)
ARSB hydrolyses C4S/C6S chains (Gallus gallus)
H2O [lysosomal lumen]
ARSB hydrolyses DS (Gallus gallus)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from DS (Gallus gallus)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Gallus gallus)
H2O [lysosomal lumen]
HYAL1 hydrolyses Chondroitin chains (Gallus gallus)
H2O [lysosomal lumen]
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Gallus gallus)
H2O [lysosomal lumen]
IDUA) hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Gallus gallus)
H2O [lysosomal lumen]
Heparan sulfate/heparin (HS-GAG) metabolism (Gallus gallus)
HS-GAG degradation (Gallus gallus)
GLB1 hydrolyses linker chain(2) (Gallus gallus)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses CS/HS precursor (Gallus gallus)
H2O [lysosomal lumen]
Heparanase (HPSE) cleaves heparan sulfate from its proteoglycan (lysosome) (Gallus gallus)
H2O [lysosomal lumen]
IDS hydrolyses Heparan sulfate chain(5) (Gallus gallus)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(1) (Gallus gallus)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(6) (Gallus gallus)
H2O [lysosomal lumen]
NAGLU hydrolyses Heparan sulfate chain(4) (Gallus gallus)
H2O [lysosomal lumen]
NAGLU hydrolyses heparan chain(2) (Gallus gallus)
H2O [lysosomal lumen]
SGSH hydrolyses Heparan sulfate chain(2) (Gallus gallus)
H2O [lysosomal lumen]
SGSH hydrolyses Heparan sulfate chain(7) (Gallus gallus)
H2O [lysosomal lumen]
Hyaluronan metabolism (Gallus gallus)
Hyaluronan uptake and degradation (Gallus gallus)
GUSB tetramer hydrolyses (HA)2 (Gallus gallus)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Gallus gallus)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from small HA fragments (Gallus gallus)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from small HA fragments (Gallus gallus)
H2O [lysosomal lumen]
HYAL1 hydrolyses (HA)50 (Gallus gallus)
H2O [lysosomal lumen]
Keratan sulfate/keratin metabolism (Gallus gallus)
Keratan sulfate degradation (Gallus gallus)
GALNS oligomer hydrolyses sulfate from Gal6S in keratan sulfate (Gallus gallus)
H2O [lysosomal lumen]
GLB1 hydrolyses a glycosaminoglycan (Gallus gallus)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from keratan sulfate (Gallus gallus)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Gallus gallus)
H2O [lysosomal lumen]
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Gallus gallus)
H2O [lysosomal lumen]
Lysosomal oligosaccharide catabolism (Gallus gallus)
MAN2B2 hydrolyses GlcNAc (Man)3 to GlcNAc:Man (Gallus gallus)
H2O [lysosomal lumen]
Metabolism of lipids (Gallus gallus)
Fatty acid metabolism (Gallus gallus)
Fatty acyl-CoA biosynthesis (Gallus gallus)
PPT1 hydrolyses palmitoylated proteins (Gallus gallus)
H2O [lysosomal lumen]
Sphingolipid metabolism (Gallus gallus)
Glycosphingolipid metabolism (Gallus gallus)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Gallus gallus)
H2O [lysosomal lumen]
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Gallus gallus)
H2O [lysosomal lumen]
Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside (Gallus gallus)
H2O [lysosomal lumen]
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Gallus gallus)
H2O [lysosomal lumen]
Beta-galactosidase hydrolyses GM1 to GM2 (Gallus gallus)
H2O [lysosomal lumen]
Both hexosaminidase A and B can cleave GalNAc from globoside (Gallus gallus)
H2O [lysosomal lumen]
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Gallus gallus)
H2O [lysosomal lumen]
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Gallus gallus)
H2O [lysosomal lumen]
Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen) (Gallus gallus)
H2O [lysosomal lumen]
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Gallus gallus)
H2O [lysosomal lumen]
Metabolism of proteins (Gallus gallus)
Post-translational protein modification (Gallus gallus)
Asparagine N-linked glycosylation (Gallus gallus)
Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein (Gallus gallus)
Synthesis of substrates in N-glycan biosythesis (Gallus gallus)
Sialic acid metabolism (Gallus gallus)
NEU4 hydrolyses Neu5Ac from glycoconjugates (Gallus gallus)
H2O [lysosomal lumen]
Transport to the Golgi and subsequent modification (Gallus gallus)
N-glycan antennae elongation in the medial/trans-Golgi (Gallus gallus)
Reactions specific to the complex N-glycan synthesis pathway (Gallus gallus)
FUCA1 hydrolyses NGP:1,6-GlcNAc (Gallus gallus)
H2O [lysosomal lumen]
Neuronal System (Gallus gallus)
Transmission across Chemical Synapses (Gallus gallus)
Neurotransmitter release cycle (Gallus gallus)
NAAA hydrolyses NAEs to FAs and ethanolamine (Gallus gallus)
H2O [lysosomal lumen]
Transport of small molecules (Gallus gallus)
Plasma lipoprotein assembly, remodeling, and clearance (Gallus gallus)
Plasma lipoprotein clearance (Gallus gallus)
LDL clearance (Gallus gallus)
LIPA hydrolyses sterol esters to sterols and fatty acids (Gallus gallus)
H2O [lysosomal lumen]
Disease (Homo sapiens)
Diseases of metabolism (Homo sapiens)
Diseases of carbohydrate metabolism (Homo sapiens)
Glycogen storage diseases (Homo sapiens)
Glycogen storage disease type II (GAA) (Homo sapiens)
Defective GAA does not hydrolyze lysosomal glycogen (Homo sapiens)
H2O [lysosomal lumen]
Mucopolysaccharidoses (Homo sapiens)
MPS I - Hurler syndrome (Homo sapiens)
Defective IDUA does not hydrolyse Heparan sulfate chain(1) (Homo sapiens)
H2O [lysosomal lumen]
Defective IDUA does not hydrolyse Heparan sulfate chain(6) (Homo sapiens)
H2O [lysosomal lumen]
Defective IDUA does not hydrolyse the unsulfated alpha-L-iduronosidic link in DS (Homo sapiens)
H2O [lysosomal lumen]
MPS II - Hunter syndrome (Homo sapiens)
Defective IDS does not hydrolyse Heparan sulfate chain(5) (Homo sapiens)
H2O [lysosomal lumen]
Defective IDS does not hydrolyse dermatan sulfate (Chebi:63517 chain) (Homo sapiens)
H2O [lysosomal lumen]
MPS IIIA - Sanfilippo syndrome A (Homo sapiens)
Defective SGSH does not hydrolyse Heparan sulfate chain(2) (Homo sapiens)
H2O [lysosomal lumen]
Defective SGSH does not hydrolyse Heparan sulfate chain(7) (Homo sapiens)
H2O [lysosomal lumen]
MPS IIIB - Sanfilippo syndrome B (Homo sapiens)
Defective NAGLU does not hydrolyse Heparan sulfate chain(4) (Homo sapiens)
H2O [lysosomal lumen]
Defective NAGLU does not hydrolyse heparan chain(2) (Homo sapiens)
H2O [lysosomal lumen]
MPS IIID - Sanfilippo syndrome D (Homo sapiens)
Defective GNS does not hydrolyse 6-sulfate from GlcNAc6S (Homo sapiens)
H2O [lysosomal lumen]
MPS IV - Morquio syndrome A (Homo sapiens)
Defective GALNS does not hydrolyse sulfate from Gal6S in keratan sulfate (Homo sapiens)
H2O [lysosomal lumen]
MPS IV - Morquio syndrome B (Homo sapiens)
Defective GLB1 does not hydrolyse a glycosaminoglycan (Homo sapiens)
H2O [lysosomal lumen]
Defective GLB1 does not hydrolyse linker chain(2) (Homo sapiens)
H2O [lysosomal lumen]
MPS IX - Natowicz syndrome (Homo sapiens)
Defective HYAL1 does not hydrolyse (HA)50 (Homo sapiens)
H2O [lysosomal lumen]
Defective HYAL1 does not hydrolyse Chondroitin chains (Homo sapiens)
H2O [lysosomal lumen]
MPS VI - Maroteaux-Lamy syndrome (Homo sapiens)
Defective ARSB does not hydrolyse C4S/C6S chains (Homo sapiens)
H2O [lysosomal lumen]
Defective ARSB does not hydrolyse DS (Homo sapiens)
H2O [lysosomal lumen]
MPS VII - Sly syndrome (Homo sapiens)
Defective GUSB does not hydrolyse (HA)2 (Homo sapiens)
H2O [lysosomal lumen]
Defective GUSB does not hydrolyse CS/HS precursor (Homo sapiens)
H2O [lysosomal lumen]
Defective GUSB does not hydrolyse GlcA-β1,3-GlcNAc (Homo sapiens)
H2O [lysosomal lumen]
Diseases of glycosylation (Homo sapiens)
Diseases associated with N-glycosylation of proteins (Homo sapiens)
Defective NEU1 causes sialidosis (Homo sapiens)
Defective NEU1 does not hydrolyse Neu5Ac from glycoconjugates (Homo sapiens)
H2O [lysosomal lumen]
Diseases associated with glycosaminoglycan metabolism (Homo sapiens)
Defective HEXA causes GM2G1 (Homo sapiens)
Defective HEXA does not cleave the terminal GalNAc from DS (Homo sapiens)
H2O [lysosomal lumen]
Defective HEXA does not cleave the terminal GalNAc from keratan sulfate (Homo sapiens)
H2O [lysosomal lumen]
Defective HEXA does not cleave the terminall GalNAc from small HA fragments (Homo sapiens)
H2O [lysosomal lumen]
Defective HEXB causes GM2G2 (Homo sapiens)
Defective HEXB does not cleave the terminal GalNAc from DS (Homo sapiens)
H2O [lysosomal lumen]
Defective HEXB does not cleave the terminal GalNAc from HA fragments (Homo sapiens)
H2O [lysosomal lumen]
Defective HEXB does not cleave the terminal GalNAc from keratan sulfate (Homo sapiens)
H2O [lysosomal lumen]
Metabolism (Homo sapiens)
Metabolism of carbohydrates (Homo sapiens)
Glycogen metabolism (Homo sapiens)
Glycogen breakdown (glycogenolysis) (Homo sapiens)
GAA hydrolyzes lysosomal glycogen (Homo sapiens)
H2O [lysosomal lumen]
Glycosaminoglycan metabolism (Homo sapiens)
Chondroitin sulfate/dermatan sulfate metabolism (Homo sapiens)
CS/DS degradation (Homo sapiens)
ARSB hydrolyses C4S/C6S chains (Homo sapiens)
H2O [lysosomal lumen]
ARSB hydrolyses DS (Homo sapiens)
H2O [lysosomal lumen]
CS is cleaved from its proteoglycan (Homo sapiens)
H2O [lysosomal lumen]
DS is cleaved from its proteoglycan (Homo sapiens)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from DS (Homo sapiens)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Homo sapiens)
H2O [lysosomal lumen]
HYAL1 hydrolyses Chondroitin chains (Homo sapiens)
H2O [lysosomal lumen]
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Homo sapiens)
H2O [lysosomal lumen]
IDUA) hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Homo sapiens)
H2O [lysosomal lumen]
Heparan sulfate/heparin (HS-GAG) metabolism (Homo sapiens)
HS-GAG degradation (Homo sapiens)
GLB1 hydrolyses linker chain(2) (Homo sapiens)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses CS/HS precursor (Homo sapiens)
H2O [lysosomal lumen]
Heparanase (HPSE) cleaves heparan sulfate from its proteoglycan (lysosome) (Homo sapiens)
H2O [lysosomal lumen]
IDS hydrolyses Heparan sulfate chain(5) (Homo sapiens)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(1) (Homo sapiens)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(6) (Homo sapiens)
H2O [lysosomal lumen]
NAGLU hydrolyses Heparan sulfate chain(4) (Homo sapiens)
H2O [lysosomal lumen]
NAGLU hydrolyses heparan chain(2) (Homo sapiens)
H2O [lysosomal lumen]
SGSH hydrolyses Heparan sulfate chain(2) (Homo sapiens)
H2O [lysosomal lumen]
SGSH hydrolyses Heparan sulfate chain(7) (Homo sapiens)
H2O [lysosomal lumen]
Hyaluronan metabolism (Homo sapiens)
Hyaluronan uptake and degradation (Homo sapiens)
GUSB tetramer hydrolyses (HA)2 (Homo sapiens)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Homo sapiens)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from small HA fragments (Homo sapiens)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from small HA fragments (Homo sapiens)
H2O [lysosomal lumen]
HYAL1 hydrolyses (HA)50 (Homo sapiens)
H2O [lysosomal lumen]
Keratan sulfate/keratin metabolism (Homo sapiens)
Keratan sulfate degradation (Homo sapiens)
GALNS oligomer hydrolyses sulfate from Gal6S in keratan sulfate (Homo sapiens)
H2O [lysosomal lumen]
GLB1 hydrolyses a glycosaminoglycan (Homo sapiens)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from keratan sulfate (Homo sapiens)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Homo sapiens)
H2O [lysosomal lumen]
Keratan sulfate is cleaved from its proteoglycan by an unknown galactosidase (Homo sapiens)
H2O [lysosomal lumen]
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Homo sapiens)
H2O [lysosomal lumen]
Lysosomal oligosaccharide catabolism (Homo sapiens)
MAN2B1 hydrolyses GlcNAc (Man)5 to GlcNAc (Man)3 (Homo sapiens)
H2O [lysosomal lumen]
MAN2B2 hydrolyses GlcNAc (Man)3 to GlcNAc:Man (Homo sapiens)
H2O [lysosomal lumen]
Metabolism of lipids (Homo sapiens)
Fatty acid metabolism (Homo sapiens)
Fatty acyl-CoA biosynthesis (Homo sapiens)
PPT1 hydrolyses palmitoylated proteins (Homo sapiens)
H2O [lysosomal lumen]
PPT2 hydrolyses PALMCoA to PALM (Homo sapiens)
H2O [lysosomal lumen]
Sphingolipid metabolism (Homo sapiens)
Glycosphingolipid metabolism (Homo sapiens)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Homo sapiens)
H2O [lysosomal lumen]
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Homo sapiens)
H2O [lysosomal lumen]
Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside (Homo sapiens)
H2O [lysosomal lumen]
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Homo sapiens)
H2O [lysosomal lumen]
Beta-galactosidase hydrolyses GM1 to GM2 (Homo sapiens)
H2O [lysosomal lumen]
Both hexosaminidase A and B can cleave GalNAc from globoside (Homo sapiens)
H2O [lysosomal lumen]
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Homo sapiens)
H2O [lysosomal lumen]
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Homo sapiens)
H2O [lysosomal lumen]
Hexosaminidase A cleaves GalNAc from GM2 to form GM3 (Homo sapiens)
H2O [lysosomal lumen]
Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen) (Homo sapiens)
H2O [lysosomal lumen]
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Homo sapiens)
H2O [lysosomal lumen]
Metabolism of proteins (Homo sapiens)
Post-translational protein modification (Homo sapiens)
Asparagine N-linked glycosylation (Homo sapiens)
Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein (Homo sapiens)
Synthesis of substrates in N-glycan biosythesis (Homo sapiens)
Sialic acid metabolism (Homo sapiens)
NEU1 hydrolyses Neu5Ac from glycoconjugates (Homo sapiens)
H2O [lysosomal lumen]
NEU4 hydrolyses Neu5Ac from glycoconjugates (Homo sapiens)
H2O [lysosomal lumen]
Transport to the Golgi and subsequent modification (Homo sapiens)
N-glycan antennae elongation in the medial/trans-Golgi (Homo sapiens)
Reactions specific to the complex N-glycan synthesis pathway (Homo sapiens)
FUCA1 hydrolyses NGP:1,6-GlcNAc (Homo sapiens)
H2O [lysosomal lumen]
Neuronal System (Homo sapiens)
Transmission across Chemical Synapses (Homo sapiens)
Neurotransmitter release cycle (Homo sapiens)
NAAA hydrolyses NAEs to FAs and ethanolamine (Homo sapiens)
H2O [lysosomal lumen]
Transport of small molecules (Homo sapiens)
Plasma lipoprotein assembly, remodeling, and clearance (Homo sapiens)
Plasma lipoprotein clearance (Homo sapiens)
LDL clearance (Homo sapiens)
LIPA hydrolyses sterol esters to sterols and fatty acids (Homo sapiens)
H2O [lysosomal lumen]
Metabolism (Mus musculus)
Metabolism of carbohydrates (Mus musculus)
Glycogen metabolism (Mus musculus)
Glycogen breakdown (glycogenolysis) (Mus musculus)
GAA hydrolyzes lysosomal glycogen (Mus musculus)
H2O [lysosomal lumen]
Glycosaminoglycan metabolism (Mus musculus)
Chondroitin sulfate/dermatan sulfate metabolism (Mus musculus)
CS/DS degradation (Mus musculus)
ARSB hydrolyses C4S/C6S chains (Mus musculus)
H2O [lysosomal lumen]
ARSB hydrolyses DS (Mus musculus)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from DS (Mus musculus)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Mus musculus)
H2O [lysosomal lumen]
HYAL1 hydrolyses Chondroitin chains (Mus musculus)
H2O [lysosomal lumen]
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Mus musculus)
H2O [lysosomal lumen]
IDUA) hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Mus musculus)
H2O [lysosomal lumen]
Heparan sulfate/heparin (HS-GAG) metabolism (Mus musculus)
HS-GAG degradation (Mus musculus)
GLB1 hydrolyses linker chain(2) (Mus musculus)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses CS/HS precursor (Mus musculus)
H2O [lysosomal lumen]
Heparanase (HPSE) cleaves heparan sulfate from its proteoglycan (lysosome) (Mus musculus)
H2O [lysosomal lumen]
IDS hydrolyses Heparan sulfate chain(5) (Mus musculus)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(1) (Mus musculus)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(6) (Mus musculus)
H2O [lysosomal lumen]
NAGLU hydrolyses Heparan sulfate chain(4) (Mus musculus)
H2O [lysosomal lumen]
NAGLU hydrolyses heparan chain(2) (Mus musculus)
H2O [lysosomal lumen]
SGSH hydrolyses Heparan sulfate chain(2) (Mus musculus)
H2O [lysosomal lumen]
SGSH hydrolyses Heparan sulfate chain(7) (Mus musculus)
H2O [lysosomal lumen]
Hyaluronan metabolism (Mus musculus)
Hyaluronan uptake and degradation (Mus musculus)
GUSB tetramer hydrolyses (HA)2 (Mus musculus)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Mus musculus)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from small HA fragments (Mus musculus)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from small HA fragments (Mus musculus)
H2O [lysosomal lumen]
HYAL1 hydrolyses (HA)50 (Mus musculus)
H2O [lysosomal lumen]
Keratan sulfate/keratin metabolism (Mus musculus)
Keratan sulfate degradation (Mus musculus)
GALNS oligomer hydrolyses sulfate from Gal6S in keratan sulfate (Mus musculus)
H2O [lysosomal lumen]
GLB1 hydrolyses a glycosaminoglycan (Mus musculus)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from keratan sulfate (Mus musculus)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Mus musculus)
H2O [lysosomal lumen]
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Mus musculus)
H2O [lysosomal lumen]
Lysosomal oligosaccharide catabolism (Mus musculus)
MAN2B1 hydrolyses GlcNAc (Man)5 to GlcNAc (Man)3 (Mus musculus)
H2O [lysosomal lumen]
MAN2B2 hydrolyses GlcNAc (Man)3 to GlcNAc:Man (Mus musculus)
H2O [lysosomal lumen]
Metabolism of lipids (Mus musculus)
Fatty acid metabolism (Mus musculus)
Fatty acyl-CoA biosynthesis (Mus musculus)
PPT1 hydrolyses palmitoylated proteins (Mus musculus)
H2O [lysosomal lumen]
PPT2 hydrolyses PALMCoA to PALM (Mus musculus)
H2O [lysosomal lumen]
Sphingolipid metabolism (Mus musculus)
Glycosphingolipid metabolism (Mus musculus)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Mus musculus)
H2O [lysosomal lumen]
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Mus musculus)
H2O [lysosomal lumen]
Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside (Mus musculus)
H2O [lysosomal lumen]
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Mus musculus)
H2O [lysosomal lumen]
Beta-galactosidase hydrolyses GM1 to GM2 (Mus musculus)
H2O [lysosomal lumen]
Both hexosaminidase A and B can cleave GalNAc from globoside (Mus musculus)
H2O [lysosomal lumen]
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Mus musculus)
H2O [lysosomal lumen]
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Mus musculus)
H2O [lysosomal lumen]
Hexosaminidase A cleaves GalNAc from GM2 to form GM3 (Mus musculus)
H2O [lysosomal lumen]
Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen) (Mus musculus)
H2O [lysosomal lumen]
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Mus musculus)
H2O [lysosomal lumen]
Metabolism of proteins (Mus musculus)
Post-translational protein modification (Mus musculus)
Asparagine N-linked glycosylation (Mus musculus)
Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein (Mus musculus)
Synthesis of substrates in N-glycan biosythesis (Mus musculus)
Sialic acid metabolism (Mus musculus)
NEU1 hydrolyses Neu5Ac from glycoconjugates (Mus musculus)
H2O [lysosomal lumen]
NEU4 hydrolyses Neu5Ac from glycoconjugates (Mus musculus)
H2O [lysosomal lumen]
Transport to the Golgi and subsequent modification (Mus musculus)
N-glycan antennae elongation in the medial/trans-Golgi (Mus musculus)
Reactions specific to the complex N-glycan synthesis pathway (Mus musculus)
FUCA1 hydrolyses NGP:1,6-GlcNAc (Mus musculus)
H2O [lysosomal lumen]
Neuronal System (Mus musculus)
Transmission across Chemical Synapses (Mus musculus)
Neurotransmitter release cycle (Mus musculus)
NAAA hydrolyses NAEs to FAs and ethanolamine (Mus musculus)
H2O [lysosomal lumen]
Transport of small molecules (Mus musculus)
Plasma lipoprotein assembly, remodeling, and clearance (Mus musculus)
Plasma lipoprotein clearance (Mus musculus)
LDL clearance (Mus musculus)
LIPA hydrolyses sterol esters to sterols and fatty acids (Mus musculus)
H2O [lysosomal lumen]
Metabolism (Rattus norvegicus)
Metabolism of carbohydrates (Rattus norvegicus)
Glycogen metabolism (Rattus norvegicus)
Glycogen breakdown (glycogenolysis) (Rattus norvegicus)
GAA hydrolyzes lysosomal glycogen (Rattus norvegicus)
H2O [lysosomal lumen]
Glycosaminoglycan metabolism (Rattus norvegicus)
Chondroitin sulfate/dermatan sulfate metabolism (Rattus norvegicus)
CS/DS degradation (Rattus norvegicus)
ARSB hydrolyses C4S/C6S chains (Rattus norvegicus)
H2O [lysosomal lumen]
ARSB hydrolyses DS (Rattus norvegicus)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from DS (Rattus norvegicus)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Rattus norvegicus)
H2O [lysosomal lumen]
HYAL1 hydrolyses Chondroitin chains (Rattus norvegicus)
H2O [lysosomal lumen]
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Rattus norvegicus)
H2O [lysosomal lumen]
IDUA) hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Rattus norvegicus)
H2O [lysosomal lumen]
Heparan sulfate/heparin (HS-GAG) metabolism (Rattus norvegicus)
HS-GAG degradation (Rattus norvegicus)
GLB1 hydrolyses linker chain(2) (Rattus norvegicus)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses CS/HS precursor (Rattus norvegicus)
H2O [lysosomal lumen]
Heparanase (HPSE) cleaves heparan sulfate from its proteoglycan (lysosome) (Rattus norvegicus)
H2O [lysosomal lumen]
IDS hydrolyses Heparan sulfate chain(5) (Rattus norvegicus)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(1) (Rattus norvegicus)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(6) (Rattus norvegicus)
H2O [lysosomal lumen]
NAGLU hydrolyses Heparan sulfate chain(4) (Rattus norvegicus)
H2O [lysosomal lumen]
NAGLU hydrolyses heparan chain(2) (Rattus norvegicus)
H2O [lysosomal lumen]
Hyaluronan metabolism (Rattus norvegicus)
Hyaluronan uptake and degradation (Rattus norvegicus)
GUSB tetramer hydrolyses (HA)2 (Rattus norvegicus)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Rattus norvegicus)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from small HA fragments (Rattus norvegicus)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from small HA fragments (Rattus norvegicus)
H2O [lysosomal lumen]
HYAL1 hydrolyses (HA)50 (Rattus norvegicus)
H2O [lysosomal lumen]
Keratan sulfate/keratin metabolism (Rattus norvegicus)
Keratan sulfate degradation (Rattus norvegicus)
GALNS oligomer hydrolyses sulfate from Gal6S in keratan sulfate (Rattus norvegicus)
H2O [lysosomal lumen]
GLB1 hydrolyses a glycosaminoglycan (Rattus norvegicus)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from keratan sulfate (Rattus norvegicus)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Rattus norvegicus)
H2O [lysosomal lumen]
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Rattus norvegicus)
H2O [lysosomal lumen]
Lysosomal oligosaccharide catabolism (Rattus norvegicus)
MAN2B1 hydrolyses GlcNAc (Man)5 to GlcNAc (Man)3 (Rattus norvegicus)
H2O [lysosomal lumen]
MAN2B2 hydrolyses GlcNAc (Man)3 to GlcNAc:Man (Rattus norvegicus)
H2O [lysosomal lumen]
Metabolism of lipids (Rattus norvegicus)
Fatty acid metabolism (Rattus norvegicus)
Fatty acyl-CoA biosynthesis (Rattus norvegicus)
PPT1 hydrolyses palmitoylated proteins (Rattus norvegicus)
H2O [lysosomal lumen]
PPT2 hydrolyses PALMCoA to PALM (Rattus norvegicus)
H2O [lysosomal lumen]
Sphingolipid metabolism (Rattus norvegicus)
Glycosphingolipid metabolism (Rattus norvegicus)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Rattus norvegicus)
H2O [lysosomal lumen]
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Rattus norvegicus)
H2O [lysosomal lumen]
Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside (Rattus norvegicus)
H2O [lysosomal lumen]
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Rattus norvegicus)
H2O [lysosomal lumen]
Beta-galactosidase hydrolyses GM1 to GM2 (Rattus norvegicus)
H2O [lysosomal lumen]
Both hexosaminidase A and B can cleave GalNAc from globoside (Rattus norvegicus)
H2O [lysosomal lumen]
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Rattus norvegicus)
H2O [lysosomal lumen]
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Rattus norvegicus)
H2O [lysosomal lumen]
Hexosaminidase A cleaves GalNAc from GM2 to form GM3 (Rattus norvegicus)
H2O [lysosomal lumen]
Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen) (Rattus norvegicus)
H2O [lysosomal lumen]
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Rattus norvegicus)
H2O [lysosomal lumen]
Metabolism of proteins (Rattus norvegicus)
Post-translational protein modification (Rattus norvegicus)
Asparagine N-linked glycosylation (Rattus norvegicus)
Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein (Rattus norvegicus)
Synthesis of substrates in N-glycan biosythesis (Rattus norvegicus)
Sialic acid metabolism (Rattus norvegicus)
NEU1 hydrolyses Neu5Ac from glycoconjugates (Rattus norvegicus)
H2O [lysosomal lumen]
NEU4 hydrolyses Neu5Ac from glycoconjugates (Rattus norvegicus)
H2O [lysosomal lumen]
Transport to the Golgi and subsequent modification (Rattus norvegicus)
N-glycan antennae elongation in the medial/trans-Golgi (Rattus norvegicus)
Reactions specific to the complex N-glycan synthesis pathway (Rattus norvegicus)
FUCA1 hydrolyses NGP:1,6-GlcNAc (Rattus norvegicus)
H2O [lysosomal lumen]
Neuronal System (Rattus norvegicus)
Transmission across Chemical Synapses (Rattus norvegicus)
Neurotransmitter release cycle (Rattus norvegicus)
NAAA hydrolyses NAEs to FAs and ethanolamine (Rattus norvegicus)
H2O [lysosomal lumen]
Transport of small molecules (Rattus norvegicus)
Plasma lipoprotein assembly, remodeling, and clearance (Rattus norvegicus)
Plasma lipoprotein clearance (Rattus norvegicus)
LDL clearance (Rattus norvegicus)
LIPA hydrolyses sterol esters to sterols and fatty acids (Rattus norvegicus)
H2O [lysosomal lumen]
Metabolism (Saccharomyces cerevisiae)
Metabolism of lipids (Saccharomyces cerevisiae)
Sphingolipid metabolism (Saccharomyces cerevisiae)
Glycosphingolipid metabolism (Saccharomyces cerevisiae)
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Saccharomyces cerevisiae)
H2O [lysosomal lumen]
Metabolism (Schizosaccharomyces pombe)
Metabolism of carbohydrates (Schizosaccharomyces pombe)
Glycogen metabolism (Schizosaccharomyces pombe)
Glycogen breakdown (glycogenolysis) (Schizosaccharomyces pombe)
GAA hydrolyzes lysosomal glycogen (Schizosaccharomyces pombe)
H2O [lysosomal lumen]
Metabolism of lipids (Schizosaccharomyces pombe)
Sphingolipid metabolism (Schizosaccharomyces pombe)
Glycosphingolipid metabolism (Schizosaccharomyces pombe)
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Schizosaccharomyces pombe)
H2O [lysosomal lumen]
Metabolism (Sus scrofa)
Metabolism of carbohydrates (Sus scrofa)
Glycogen metabolism (Sus scrofa)
Glycogen breakdown (glycogenolysis) (Sus scrofa)
GAA hydrolyzes lysosomal glycogen (Sus scrofa)
H2O [lysosomal lumen]
Glycosaminoglycan metabolism (Sus scrofa)
Chondroitin sulfate/dermatan sulfate metabolism (Sus scrofa)
CS/DS degradation (Sus scrofa)
ARSB hydrolyses C4S/C6S chains (Sus scrofa)
H2O [lysosomal lumen]
ARSB hydrolyses DS (Sus scrofa)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from DS (Sus scrofa)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Sus scrofa)
H2O [lysosomal lumen]
HYAL1 hydrolyses Chondroitin chains (Sus scrofa)
H2O [lysosomal lumen]
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Sus scrofa)
H2O [lysosomal lumen]
IDUA) hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Sus scrofa)
H2O [lysosomal lumen]
Heparan sulfate/heparin (HS-GAG) metabolism (Sus scrofa)
HS-GAG degradation (Sus scrofa)
GLB1 hydrolyses linker chain(2) (Sus scrofa)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses CS/HS precursor (Sus scrofa)
H2O [lysosomal lumen]
Heparanase (HPSE) cleaves heparan sulfate from its proteoglycan (lysosome) (Sus scrofa)
H2O [lysosomal lumen]
IDS hydrolyses Heparan sulfate chain(5) (Sus scrofa)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(1) (Sus scrofa)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(6) (Sus scrofa)
H2O [lysosomal lumen]
NAGLU hydrolyses Heparan sulfate chain(4) (Sus scrofa)
H2O [lysosomal lumen]
NAGLU hydrolyses heparan chain(2) (Sus scrofa)
H2O [lysosomal lumen]
SGSH hydrolyses Heparan sulfate chain(2) (Sus scrofa)
H2O [lysosomal lumen]
SGSH hydrolyses Heparan sulfate chain(7) (Sus scrofa)
H2O [lysosomal lumen]
Hyaluronan metabolism (Sus scrofa)
Hyaluronan uptake and degradation (Sus scrofa)
GUSB tetramer hydrolyses (HA)2 (Sus scrofa)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Sus scrofa)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from small HA fragments (Sus scrofa)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from small HA fragments (Sus scrofa)
H2O [lysosomal lumen]
HYAL1 hydrolyses (HA)50 (Sus scrofa)
H2O [lysosomal lumen]
Keratan sulfate/keratin metabolism (Sus scrofa)
Keratan sulfate degradation (Sus scrofa)
GALNS oligomer hydrolyses sulfate from Gal6S in keratan sulfate (Sus scrofa)
H2O [lysosomal lumen]
GLB1 hydrolyses a glycosaminoglycan (Sus scrofa)
H2O [lysosomal lumen]
HEXA cleaves the terminal GalNAc from keratan sulfate (Sus scrofa)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Sus scrofa)
H2O [lysosomal lumen]
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Sus scrofa)
H2O [lysosomal lumen]
Lysosomal oligosaccharide catabolism (Sus scrofa)
MAN2B1 hydrolyses GlcNAc (Man)5 to GlcNAc (Man)3 (Sus scrofa)
H2O [lysosomal lumen]
MAN2B2 hydrolyses GlcNAc (Man)3 to GlcNAc:Man (Sus scrofa)
H2O [lysosomal lumen]
Metabolism of lipids (Sus scrofa)
Fatty acid metabolism (Sus scrofa)
Fatty acyl-CoA biosynthesis (Sus scrofa)
PPT1 hydrolyses palmitoylated proteins (Sus scrofa)
H2O [lysosomal lumen]
PPT2 hydrolyses PALMCoA to PALM (Sus scrofa)
H2O [lysosomal lumen]
Sphingolipid metabolism (Sus scrofa)
Glycosphingolipid metabolism (Sus scrofa)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Sus scrofa)
H2O [lysosomal lumen]
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Sus scrofa)
H2O [lysosomal lumen]
Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside (Sus scrofa)
H2O [lysosomal lumen]
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Sus scrofa)
H2O [lysosomal lumen]
Beta-galactosidase hydrolyses GM1 to GM2 (Sus scrofa)
H2O [lysosomal lumen]
Both hexosaminidase A and B can cleave GalNAc from globoside (Sus scrofa)
H2O [lysosomal lumen]
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Sus scrofa)
H2O [lysosomal lumen]
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Sus scrofa)
H2O [lysosomal lumen]
Hexosaminidase A cleaves GalNAc from GM2 to form GM3 (Sus scrofa)
H2O [lysosomal lumen]
Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen) (Sus scrofa)
H2O [lysosomal lumen]
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Sus scrofa)
H2O [lysosomal lumen]
Metabolism of proteins (Sus scrofa)
Post-translational protein modification (Sus scrofa)
Asparagine N-linked glycosylation (Sus scrofa)
Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein (Sus scrofa)
Synthesis of substrates in N-glycan biosythesis (Sus scrofa)
Sialic acid metabolism (Sus scrofa)
NEU1 hydrolyses Neu5Ac from glycoconjugates (Sus scrofa)
H2O [lysosomal lumen]
NEU4 hydrolyses Neu5Ac from glycoconjugates (Sus scrofa)
H2O [lysosomal lumen]
Transport to the Golgi and subsequent modification (Sus scrofa)
N-glycan antennae elongation in the medial/trans-Golgi (Sus scrofa)
Reactions specific to the complex N-glycan synthesis pathway (Sus scrofa)
FUCA1 hydrolyses NGP:1,6-GlcNAc (Sus scrofa)
H2O [lysosomal lumen]
Neuronal System (Sus scrofa)
Transmission across Chemical Synapses (Sus scrofa)
Neurotransmitter release cycle (Sus scrofa)
NAAA hydrolyses NAEs to FAs and ethanolamine (Sus scrofa)
H2O [lysosomal lumen]
Transport of small molecules (Sus scrofa)
Plasma lipoprotein assembly, remodeling, and clearance (Sus scrofa)
Plasma lipoprotein clearance (Sus scrofa)
LDL clearance (Sus scrofa)
LIPA hydrolyses sterol esters to sterols and fatty acids (Sus scrofa)
H2O [lysosomal lumen]
Metabolism (Xenopus tropicalis)
Metabolism of carbohydrates (Xenopus tropicalis)
Glycogen metabolism (Xenopus tropicalis)
Glycogen breakdown (glycogenolysis) (Xenopus tropicalis)
GAA hydrolyzes lysosomal glycogen (Xenopus tropicalis)
H2O [lysosomal lumen]
Glycosaminoglycan metabolism (Xenopus tropicalis)
Chondroitin sulfate/dermatan sulfate metabolism (Xenopus tropicalis)
CS/DS degradation (Xenopus tropicalis)
ARSB hydrolyses C4S/C6S chains (Xenopus tropicalis)
H2O [lysosomal lumen]
ARSB hydrolyses DS (Xenopus tropicalis)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from DS (Xenopus tropicalis)
H2O [lysosomal lumen]
HYAL1 hydrolyses Chondroitin chains (Xenopus tropicalis)
H2O [lysosomal lumen]
IDS hydrolyses dermatan sulfate (Chebi:63517 chain) (Xenopus tropicalis)
H2O [lysosomal lumen]
IDUA) hydrolyses the unsulfated alpha-L-iduronosidic link in DS (Xenopus tropicalis)
H2O [lysosomal lumen]
Heparan sulfate/heparin (HS-GAG) metabolism (Xenopus tropicalis)
HS-GAG degradation (Xenopus tropicalis)
GLB1 hydrolyses linker chain(2) (Xenopus tropicalis)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses CS/HS precursor (Xenopus tropicalis)
H2O [lysosomal lumen]
Heparanase (HPSE) cleaves heparan sulfate from its proteoglycan (lysosome) (Xenopus tropicalis)
H2O [lysosomal lumen]
IDS hydrolyses Heparan sulfate chain(5) (Xenopus tropicalis)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(1) (Xenopus tropicalis)
H2O [lysosomal lumen]
IDUA hydrolyses Heparan sulfate chain(6) (Xenopus tropicalis)
H2O [lysosomal lumen]
SGSH hydrolyses Heparan sulfate chain(2) (Xenopus tropicalis)
H2O [lysosomal lumen]
SGSH hydrolyses Heparan sulfate chain(7) (Xenopus tropicalis)
H2O [lysosomal lumen]
Hyaluronan metabolism (Xenopus tropicalis)
Hyaluronan uptake and degradation (Xenopus tropicalis)
GUSB tetramer hydrolyses (HA)2 (Xenopus tropicalis)
H2O [lysosomal lumen]
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Xenopus tropicalis)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from small HA fragments (Xenopus tropicalis)
H2O [lysosomal lumen]
HYAL1 hydrolyses (HA)50 (Xenopus tropicalis)
H2O [lysosomal lumen]
Keratan sulfate/keratin metabolism (Xenopus tropicalis)
Keratan sulfate degradation (Xenopus tropicalis)
GALNS oligomer hydrolyses sulfate from Gal6S in keratan sulfate (Xenopus tropicalis)
H2O [lysosomal lumen]
GLB1 hydrolyses a glycosaminoglycan (Xenopus tropicalis)
H2O [lysosomal lumen]
HEXB cleaves the terminal GalNAc from keratan sulfate (Xenopus tropicalis)
H2O [lysosomal lumen]
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate (Xenopus tropicalis)
H2O [lysosomal lumen]
Lysosomal oligosaccharide catabolism (Xenopus tropicalis)
MAN2B1 hydrolyses GlcNAc (Man)5 to GlcNAc (Man)3 (Xenopus tropicalis)
H2O [lysosomal lumen]
MAN2B2 hydrolyses GlcNAc (Man)3 to GlcNAc:Man (Xenopus tropicalis)
H2O [lysosomal lumen]
Metabolism of lipids (Xenopus tropicalis)
Fatty acid metabolism (Xenopus tropicalis)
Fatty acyl-CoA biosynthesis (Xenopus tropicalis)
PPT1 hydrolyses palmitoylated proteins (Xenopus tropicalis)
H2O [lysosomal lumen]
PPT2 hydrolyses PALMCoA to PALM (Xenopus tropicalis)
H2O [lysosomal lumen]
Sphingolipid metabolism (Xenopus tropicalis)
Glycosphingolipid metabolism (Xenopus tropicalis)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Xenopus tropicalis)
H2O [lysosomal lumen]
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Xenopus tropicalis)
H2O [lysosomal lumen]
Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside (Xenopus tropicalis)
H2O [lysosomal lumen]
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Xenopus tropicalis)
H2O [lysosomal lumen]
Beta-galactosidase hydrolyses GM1 to GM2 (Xenopus tropicalis)
H2O [lysosomal lumen]
Both hexosaminidase A and B can cleave GalNAc from globoside (Xenopus tropicalis)
H2O [lysosomal lumen]
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Xenopus tropicalis)
H2O [lysosomal lumen]
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Xenopus tropicalis)
H2O [lysosomal lumen]
Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen) (Xenopus tropicalis)
H2O [lysosomal lumen]
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Xenopus tropicalis)
H2O [lysosomal lumen]
Metabolism of proteins (Xenopus tropicalis)
Post-translational protein modification (Xenopus tropicalis)
Asparagine N-linked glycosylation (Xenopus tropicalis)
Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein (Xenopus tropicalis)
Synthesis of substrates in N-glycan biosythesis (Xenopus tropicalis)
Sialic acid metabolism (Xenopus tropicalis)
NEU1 hydrolyses Neu5Ac from glycoconjugates (Xenopus tropicalis)
H2O [lysosomal lumen]
NEU4 hydrolyses Neu5Ac from glycoconjugates (Xenopus tropicalis)
H2O [lysosomal lumen]
Transport to the Golgi and subsequent modification (Xenopus tropicalis)
N-glycan antennae elongation in the medial/trans-Golgi (Xenopus tropicalis)
Reactions specific to the complex N-glycan synthesis pathway (Xenopus tropicalis)
FUCA1 hydrolyses NGP:1,6-GlcNAc (Xenopus tropicalis)
H2O [lysosomal lumen]
Neuronal System (Xenopus tropicalis)
Transmission across Chemical Synapses (Xenopus tropicalis)
Neurotransmitter release cycle (Xenopus tropicalis)
NAAA hydrolyses NAEs to FAs and ethanolamine (Xenopus tropicalis)
H2O [lysosomal lumen]
External Reference Information
External Reference
water [ChEBI:15377]
Participates
as an input of
PPT2 hydrolyses PALMCoA to PALM (Dictyostelium discoideum)
PPT2 hydrolyses PALMCoA to PALM (Drosophila melanogaster)
PPT2 hydrolyses PALMCoA to PALM (Xenopus tropicalis)
PPT2 hydrolyses PALMCoA to PALM (Danio rerio)
PPT2 hydrolyses PALMCoA to PALM (Sus scrofa)
PPT2 hydrolyses PALMCoA to PALM (Bos taurus)
PPT2 hydrolyses PALMCoA to PALM (Canis familiaris)
PPT2 hydrolyses PALMCoA to PALM (Rattus norvegicus)
PPT2 hydrolyses PALMCoA to PALM (Mus musculus)
PPT2 hydrolyses PALMCoA to PALM (Homo sapiens)
PPT1 hydrolyses palmitoylated proteins (Dictyostelium discoideum)
PPT1 hydrolyses palmitoylated proteins (Caenorhabditis elegans)
PPT1 hydrolyses palmitoylated proteins (Drosophila melanogaster)
PPT1 hydrolyses palmitoylated proteins (Gallus gallus)
PPT1 hydrolyses palmitoylated proteins (Xenopus tropicalis)
PPT1 hydrolyses palmitoylated proteins (Danio rerio)
PPT1 hydrolyses palmitoylated proteins (Sus scrofa)
PPT1 hydrolyses palmitoylated proteins (Bos taurus)
PPT1 hydrolyses palmitoylated proteins (Canis familiaris)
PPT1 hydrolyses palmitoylated proteins (Rattus norvegicus)
PPT1 hydrolyses palmitoylated proteins (Mus musculus)
PPT1 hydrolyses palmitoylated proteins (Homo sapiens)
Defective IDUA does not hydrolyse Heparan sulfate chain(1) (Homo sapiens)
Defective IDUA does not hydrolyse Heparan sulfate chain(6) (Homo sapiens)
Defective IDUA does not hydrolyse the unsulfated alpha-L-iduronosidic link in DS (Homo sapiens)
Defective ARSB does not hydrolyse C4S/C6S chains (Homo sapiens)
Defective ARSB does not hydrolyse DS (Homo sapiens)
Defective NAGLU does not hydrolyse heparan chain(2) (Homo sapiens)
Defective NAGLU does not hydrolyse Heparan sulfate chain(4) (Homo sapiens)
Defective HYAL1 does not hydrolyse Chondroitin chains (Homo sapiens)
Defective HYAL1 does not hydrolyse (HA)50 (Homo sapiens)
Defective IDS does not hydrolyse Heparan sulfate chain(5) (Homo sapiens)
Defective IDS does not hydrolyse dermatan sulfate (Chebi:63517 chain) (Homo sapiens)
Defective GUSB does not hydrolyse CS/HS precursor (Homo sapiens)
Defective GUSB does not hydrolyse GlcA-β1,3-GlcNAc (Homo sapiens)
Defective GUSB does not hydrolyse (HA)2 (Homo sapiens)
Defective GLB1 does not hydrolyse a glycosaminoglycan (Homo sapiens)
Defective GLB1 does not hydrolyse linker chain(2) (Homo sapiens)
Defective GALNS does not hydrolyse sulfate from Gal6S in keratan sulfate (Homo sapiens)
Defective SGSH does not hydrolyse Heparan sulfate chain(2) (Homo sapiens)
Defective SGSH does not hydrolyse Heparan sulfate chain(7) (Homo sapiens)
Defective GNS does not hydrolyse 6-sulfate from GlcNAc6S (Homo sapiens)
Defective GAA does not hydrolyze lysosomal glycogen (Homo sapiens)
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Dictyostelium discoideum)
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Caenorhabditis elegans)
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Drosophila melanogaster)
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Gallus gallus)
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Xenopus tropicalis)
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Danio rerio)
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Sus scrofa)
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Bos taurus)
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Canis familiaris)
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Rattus norvegicus)
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Mus musculus)
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Dictyostelium discoideum)
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Gallus gallus)
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Xenopus tropicalis)
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Danio rerio)
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Sus scrofa)
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Bos taurus)
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Canis familiaris)
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Rattus norvegicus)
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Mus musculus)
Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers (Homo sapiens)
Beta-galactosidase can also hydrolyse globosides to form cerebrosides (Homo sapiens)
Both hexosaminidase A and B can cleave GalNAc from globoside (Dictyostelium discoideum)
Both hexosaminidase A and B can cleave GalNAc from globoside (Caenorhabditis elegans)
Both hexosaminidase A and B can cleave GalNAc from globoside (Drosophila melanogaster)
Both hexosaminidase A and B can cleave GalNAc from globoside (Gallus gallus)
Both hexosaminidase A and B can cleave GalNAc from globoside (Xenopus tropicalis)
Both hexosaminidase A and B can cleave GalNAc from globoside (Danio rerio)
Both hexosaminidase A and B can cleave GalNAc from globoside (Sus scrofa)
Both hexosaminidase A and B can cleave GalNAc from globoside (Bos taurus)
Both hexosaminidase A and B can cleave GalNAc from globoside (Rattus norvegicus)
Both hexosaminidase A and B can cleave GalNAc from globoside (Mus musculus)
Both hexosaminidase A and B can cleave GalNAc from globoside (Homo sapiens)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Caenorhabditis elegans)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Gallus gallus)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Xenopus tropicalis)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Danio rerio)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Sus scrofa)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Bos taurus)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Canis familiaris)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Rattus norvegicus)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Mus musculus)
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Saccharomyces cerevisiae)
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Schizosaccharomyces pombe)
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Dictyostelium discoideum)
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Caenorhabditis elegans)
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Drosophila melanogaster)
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Gallus gallus)
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Xenopus tropicalis)
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Danio rerio)
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Sus scrofa)
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Bos taurus)
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Canis familiaris)
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Rattus norvegicus)
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Mus musculus)
Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome) (Homo sapiens)
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Caenorhabditis elegans)
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Gallus gallus)
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Xenopus tropicalis)
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Danio rerio)
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Sus scrofa)
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Bos taurus)
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Canis familiaris)
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Rattus norvegicus)
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Mus musculus)
Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide (Homo sapiens)
Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome) (Homo sapiens)
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Caenorhabditis elegans)
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Drosophila melanogaster)
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Gallus gallus)
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Xenopus tropicalis)
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Danio rerio)
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Sus scrofa)
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Bos taurus)
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Canis familiaris)
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Rattus norvegicus)
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Mus musculus)
Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Homo sapiens)
Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside (Gallus gallus)
Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside (Xenopus tropicalis)
Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside (Danio rerio)
Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside (Sus scrofa)
Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside (Bos taurus)
Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside (Canis familiaris)
Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside (Rattus norvegicus)
Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside (Mus musculus)
Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside (Homo sapiens)
Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen) (Gallus gallus)
Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen) (Xenopus tropicalis)
Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen) (Danio rerio)
Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen) (Sus scrofa)
Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen) (Bos taurus)
Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen) (Canis familiaris)
Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen) (Rattus norvegicus)
Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen) (Mus musculus)
Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen) (Homo sapiens)
Hexosaminidase A cleaves GalNAc from GM2 to form GM3 (Caenorhabditis elegans)
Hexosaminidase A cleaves GalNAc from GM2 to form GM3 (Danio rerio)
Hexosaminidase A cleaves GalNAc from GM2 to form GM3 (Sus scrofa)
Hexosaminidase A cleaves GalNAc from GM2 to form GM3 (Bos taurus)
Hexosaminidase A cleaves GalNAc from GM2 to form GM3 (Rattus norvegicus)
Hexosaminidase A cleaves GalNAc from GM2 to form GM3 (Mus musculus)
Beta-galactosidase hydrolyses GM1 to GM2 (Dictyostelium discoideum)
Beta-galactosidase hydrolyses GM1 to GM2 (Caenorhabditis elegans)
Beta-galactosidase hydrolyses GM1 to GM2 (Drosophila melanogaster)
Beta-galactosidase hydrolyses GM1 to GM2 (Gallus gallus)
Beta-galactosidase hydrolyses GM1 to GM2 (Xenopus tropicalis)
Beta-galactosidase hydrolyses GM1 to GM2 (Danio rerio)
Beta-galactosidase hydrolyses GM1 to GM2 (Sus scrofa)
Beta-galactosidase hydrolyses GM1 to GM2 (Bos taurus)
Beta-galactosidase hydrolyses GM1 to GM2 (Canis familiaris)
Beta-galactosidase hydrolyses GM1 to GM2 (Rattus norvegicus)
Beta-galactosidase hydrolyses GM1 to GM2 (Mus musculus)
Beta-galactosidase hydrolyses GM1 to GM2 (Homo sapiens)
Hexosaminidase A cleaves GalNAc from GM2 to form GM3 (Homo sapiens)
Defective NEU1 does not hydrolyse Neu5Ac from glycoconjugates (Homo sapiens)
Defective HEXA does not cleave the terminal GalNAc from DS (Homo sapiens)
Defective HEXA does not cleave the terminal GalNAc from keratan sulfate (Homo sapiens)
Defective HEXA does not cleave the terminall GalNAc from small HA fragments (Homo sapiens)
Defective HEXB does not cleave the terminal GalNAc from DS (Homo sapiens)
Defective HEXB does not cleave the terminal GalNAc from HA fragments (Homo sapiens)
Defective HEXB does not cleave the terminal GalNAc from keratan sulfate (Homo sapiens)
LIPA hydrolyses sterol esters to sterols and fatty acids (Gallus gallus)
LIPA hydrolyses sterol esters to sterols and fatty acids (Danio rerio)
LIPA hydrolyses sterol esters to sterols and fatty acids (Sus scrofa)
LIPA hydrolyses sterol esters to sterols and fatty acids (Bos taurus)
LIPA hydrolyses sterol esters to sterols and fatty acids (Canis familiaris)
LIPA hydrolyses sterol esters to sterols and fatty acids (Rattus norvegicus)
LIPA hydrolyses sterol esters to sterols and fatty acids (Mus musculus)
LIPA hydrolyses sterol esters to sterols and fatty acids (Homo sapiens)
NAAA hydrolyses NAEs to FAs and ethanolamine (Caenorhabditis elegans)
NAAA hydrolyses NAEs to FAs and ethanolamine (Gallus gallus)
NAAA hydrolyses NAEs to FAs and ethanolamine (Xenopus tropicalis)
NAAA hydrolyses NAEs to FAs and ethanolamine (Sus scrofa)
NAAA hydrolyses NAEs to FAs and ethanolamine (Bos taurus)
NAAA hydrolyses NAEs to FAs and ethanolamine (Rattus norvegicus)
NAAA hydrolyses NAEs to FAs and ethanolamine (Mus musculus)
NAAA hydrolyses NAEs to FAs and ethanolamine (Homo sapiens)
GAA hydrolyzes lysosomal glycogen (Schizosaccharomyces pombe)
GAA hydrolyzes lysosomal glycogen (Xenopus tropicalis)
GAA hydrolyzes lysosomal glycogen (Sus scrofa)
GAA hydrolyzes lysosomal glycogen (Bos taurus)
GAA hydrolyzes lysosomal glycogen (Rattus norvegicus)
GAA hydrolyzes lysosomal glycogen (Mus musculus)
GAA hydrolyzes lysosomal glycogen (Homo sapiens)
HEXB cleaves the terminal GalNAc from small HA fragments (Dictyostelium discoideum)
HEXB cleaves the terminal GalNAc from small HA fragments (Caenorhabditis elegans)
HEXB cleaves the terminal GalNAc from small HA fragments (Drosophila melanogaster)
HEXB cleaves the terminal GalNAc from small HA fragments (Gallus gallus)
HEXB cleaves the terminal GalNAc from small HA fragments (Xenopus tropicalis)
HEXB cleaves the terminal GalNAc from small HA fragments (Danio rerio)
HEXB cleaves the terminal GalNAc from small HA fragments (Sus scrofa)
HEXB cleaves the terminal GalNAc from small HA fragments (Bos taurus)
HEXB cleaves the terminal GalNAc from small HA fragments (Rattus norvegicus)
HEXB cleaves the terminal GalNAc from small HA fragments (Mus musculus)
HEXB cleaves the terminal GalNAc from small HA fragments (Homo sapiens)
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Caenorhabditis elegans)
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Drosophila melanogaster)
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Gallus gallus)
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Xenopus tropicalis)
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Danio rerio)
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Sus scrofa)
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Bos taurus)
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Canis familiaris)
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Rattus norvegicus)
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Mus musculus)
GUSB tetramer hydrolyses GlcA-β1,3-GlcNAc (Homo sapiens)
HEXA cleaves the terminal GalNAc from small HA fragments (Dictyostelium discoideum)
HEXA cleaves the terminal GalNAc from small HA fragments (Caenorhabditis elegans)
HEXA cleaves the terminal GalNAc from small HA fragments (Drosophila melanogaster)
HEXA cleaves the terminal GalNAc from small HA fragments (Gallus gallus)
HEXA cleaves the terminal GalNAc from small HA fragments (Danio rerio)
HEXA cleaves the terminal GalNAc from small HA fragments (Sus scrofa)
HEXA cleaves the terminal GalNAc from small HA fragments (Bos taurus)
HEXA cleaves the terminal GalNAc from small HA fragments (Rattus norvegicus)
HEXA cleaves the terminal GalNAc from small HA fragments (Mus musculus)
GUSB tetramer hydrolyses (HA)2 (Caenorhabditis elegans)
GUSB tetramer hydrolyses (HA)2 (Drosophila melanogaster)
GUSB tetramer hydrolyses (HA)2 (Gallus gallus)
GUSB tetramer hydrolyses (HA)2 (Xenopus tropicalis)
GUSB tetramer hydrolyses (HA)2 (Danio rerio)
GUSB tetramer hydrolyses (HA)2 (Sus scrofa)
GUSB tetramer hydrolyses (HA)2 (Bos taurus)
GUSB tetramer hydrolyses (HA)2 (Canis familiaris)
GUSB tetramer hydrolyses (HA)2 (Rattus norvegicus)
GUSB tetramer hydrolyses (HA)2 (Mus musculus)
HYAL1 hydrolyses (HA)50 (Caenorhabditis elegans)
HYAL1 hydrolyses (HA)50 (Gallus gallus)
HYAL1 hydrolyses (HA)50 (Xenopus tropicalis)
HYAL1 hydrolyses (HA)50 (Danio rerio)
HYAL1 hydrolyses (HA)50 (Sus scrofa)
HYAL1 hydrolyses (HA)50 (Bos taurus)