Search results for ARSA

Showing 16 results out of 19

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Protein (7 results from a total of 10)

Identifier: R-HSA-1614334
Species: Homo sapiens
Compartment: endoplasmic reticulum lumen
Primary external reference: UniProt: ARSA: P15289
Identifier: R-HSA-6798776
Species: Homo sapiens
Compartment: azurophil granule lumen
Primary external reference: UniProt: ARSA: P15289
Identifier: R-HSA-6798775
Species: Homo sapiens
Compartment: azurophil granule lumen
Primary external reference: UniProt: ARSA: P15289
Identifier: R-HSA-508497
Species: Homo sapiens
Compartment: lysosomal lumen
Primary external reference: UniProt: ARSA: P15289
Identifier: R-HSA-6806192
Species: Homo sapiens
Compartment: extracellular region
Primary external reference: UniProt: P15289
Identifier: R-HSA-1614307
Species: Homo sapiens
Compartment: endoplasmic reticulum lumen
Primary external reference: UniProt: P15289
Identifier: R-HSA-1606798
Species: Homo sapiens
Compartment: lysosomal lumen
Primary external reference: UniProt: P15289

Complex (3 results from a total of 3)

Identifier: R-HSA-1614339
Species: Homo sapiens
Compartment: endoplasmic reticulum lumen
Identifier: R-HSA-1614355
Species: Homo sapiens
Compartment: endoplasmic reticulum lumen
Identifier: R-HSA-1606814
Species: Homo sapiens
Compartment: lysosomal lumen

Reaction (5 results from a total of 5)

Identifier: R-HSA-2248891
Species: Homo sapiens
Compartment: endoplasmic reticulum lumen, lysosomal lumen
Activated arylsulfatase A (ARSA) translocates to lysosomes by an unknown mechanism (see review von Figura et al. 1998).
Identifier: R-HSA-1606807
Species: Homo sapiens
Compartment: lysosomal lumen
Arylsulfatase A (ARSA) (Stein et al. 1989) hydrolyses a sulfatide (a cerebroside 3-sulfate) to form a cerebroside and sulfate. ARSA is present in the lysosomal lumen and comprises two chains, component B and C linked by disulphide bonds (Fujii et al. 1992). The conversion to 3-oxoalanine (formylglycine, FGly) of a cysteine residue is critical for catalytic activity in all eukaryotes (Chruszcz et al. 2003, Lukatela et al. 1998).
Defects in ARSA are a cause of leukodystrophy metachromatic (MLD) (MIM:250100), characterized by lysosomal storage of cerebroside-3-sulfate in neural and non-neural tissues (Gieselmann et al. 1991, Polten et al. 1991). Arylsulfatase A activity is reduced in multiple sulfatase deficiency (MSD) (MIM:272200), a disorder characterized by decreased activity of sulfatases. The defect is due to the lack of post-translational modification of the critical cysteine needed for activity (Schmidt et al. 1995).
Identifier: R-HSA-1630304
Species: Homo sapiens
Compartment: lysosomal lumen
N-acetylgalactosamine 6-sulfate sulfatase (GALNS) hydrolyses sulfate from galactose 6-sulfate units of keratan sulfate (KS, shown here) and sulfate from N-acetyl-D-galactosamine 6-sulfate units of chondroitin sulfate (CS, not shown) (Lim & Horwitz 1981, Masue et al. 1991). The conversion to 3-oxoalanine (C-formylglycine, FGly) of a cysteine residue in eukaryotes, is critical for catalytic activity, based on similarity to the prototypical arylsulfatase ARSA (Chruszcz et al. 2003, Lukatela et al. 1998). Defects in GALNS cause mucopolysaccharidosis type IVA (MPSIVA, MIM:253000), also called Morquio A syndrome, a lysosomal storage disease characterized by intracellular accumulation of KS and CS (Fukuda et al. 1992).
Identifier: R-HSA-1793207
Species: Homo sapiens
Compartment: lysosomal lumen
Arylsulfatase B using calcium cofactor (ARSB:Ca2+) hydrolyses sulfate from N-acetylgalactosamine 4-sulfate (or 6-sulfate) units (GalNAc 4-sulfate or GalNAc 6-sulfate) within chondroitin sulfate (Gorham & Cantz 1978). The conversion to 3-oxoalanine (formylglycine, FGly) of a cysteine residue in eukaryotes, is critical for catalytic activity, based on similarity to the prototypical arylsulfatase ARSA (Chruszcz et al. 2003, Lukatela et al. 1998). Defects in ARSB are the cause of mucopolysaccharidosis type VI (MPSVI) (MIM:253200, also called Maroteaux-Lamy syndrome (Wicker et al. 1991). ARSB activity is defective in multiple sulfatase deficiency (MSD) (MIM:272200) (Schmidt et al. 1995).
Identifier: R-HSA-1606789
Species: Homo sapiens
Compartment: lysosomal lumen
Arylsulfatase B (ARSB) hydrolyses sulfate from N-acetylgalactosamine 4-sulfate units within dermatan sulfate (DS; Gorham & Cantz 1978). The conversion to 3-oxoalanine (formylglycine, FGly) of a cysteine residue in eukaryotes, is critical for catalytic activity, based on similarity to the prototypical arylsulfatase ARSA (Chruszcz et al. 2003, Lukatela et al. 1998). Defects in ARSB are the cause of mucopolysaccharidosis type VI (MPSVI) (MIM:253200, also called Maroteaux-Lamy syndrome (Wicker et al. 1991). ARSB activity is defective in multiple sulfatase deficiency (MSD) (MIM:272200) (Schmidt et al. 1995).

Icon (1 results from a total of 1)

Species: Homo sapiens
Curator: Bruce May
Designer: Cristoffer Sevilla
ASNA1 icon
ATPase ASNA1
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