Search results for PSAP

Showing 21 results out of 61

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Protein (4 results from a total of 21)

Identifier: R-HSA-9840325
Species: Homo sapiens
Compartment: lysosomal lumen
Primary external reference: UniProt: PSAP: P07602
Identifier: R-HSA-5336190
Species: Homo sapiens
Compartment: extracellular region
Primary external reference: UniProt: PSAP: P07602
TXLIDNNATEEILY where X is a D-alanine
Identifier: R-HSA-6799212
Species: Homo sapiens
Compartment: azurophil granule membrane
Primary external reference: UniProt: PSAP: P07602
Identifier: R-HSA-6806262
Species: Homo sapiens
Compartment: plasma membrane
Primary external reference: UniProt: PSAP: P07602

Reaction (4 results from a total of 13)

Identifier: R-HSA-9840334
Species: Homo sapiens
Compartment: lysosomal lumen, lysosomal membrane
After its transport to lysosomes, prosaposin (PSAP) gets cleaved by unknown proteases into the fragments PSAP(60-142) (Saposin A), PSAP(195-273) (Saposin B), PSAP(311-391) (Saposin C) and PSAP(405-486) (Saposin D). Intermediate polyproteins during cleavage appear to be B-C-D, B-C, and C-D (Furst et al., 1988; Leonova et al., 1996; saposins were reviewed by O'Brien & Kishimoto, 1991; Morales et al., 1995; Kolter et al., 2005: Kolter & Sandhoff, 2010; Garrido-Arandia et al., 2018). Bioinformatics suggests that different peptidases, including trypsin, may be responsible for cleavage at different PSAP cleavage sites. In its dimerized form PSAP(195-273) (Saposin B) is centrally involved in mobilizing glycosphingolipids for catabolism, while PSAP(60-142) (Saposin A) and PSAP(405-486) (Saposin D) activate GALC and ASAH1, respectively.
Identifier: R-HSA-9840488
Species: Homo sapiens
Compartment: lysosomal lumen
Saposin B (PSAP(195-273)) is the only PSAP fragment that forms a dimer. The dimer is disulfide-linked and can switch between two conformations, one of which shows an open hydrophobic pocket, supporting a lipid solubilization mechanism for saposin B, consistent with the observation that saposin B can act as a lipid-transport protein (Vaccaro et al., 1995; Ahn et al., 2002; Ahn et al., 2003).
Identifier: R-HSA-9842115
Species: Homo sapiens
Compartment: lysosomal lumen, multivesicular body, internal vesicle membrane
By encapsulating phosphatidylethanolamines (PE) of different sizes from the membrane of intralysosomal luminal vesicles (ILV), the Saposin B (PSAP(195-273) dimer can solubilize relatively large lipid ligands from these membranes (Ahn et al., 2003).
Identifier: R-HSA-1605736
Species: Homo sapiens
Compartment: lysosomal lumen
Alpha-galactosidase A (GLA) (Bishop et al. 1986) removes the terminal galactose residue from glycolipids or glycoproteins, mobilized by PSAP(195-273) (Saposin B), resulting in galactose and an alcohol. An example is the Fabry disease substrate globotriaosylceramide (Gb3Cer) which is hydrolysed to form galactose and lactosylceramide. GLA functions as a homodimer (Garman & Garboczi 2004) and defects in this enzyme lead to Fabry disease (FD) (MIM:301500), a rare X-linked sphingolipidosis disease where glycolipids such as Gb3Cer accumulate in many tissues (Garman & Garboczi 2004, Eng et al. 1993, Shabeer et al. 2006). Researchers also observed elevated Gb3Cer in prosaposin deficiency (PSAPD, MIM: 611721) cases, a rare disease with low levels of all saposins. Saposin B (PSAP(195-273)) is an essential cofactor to the reaction (Bradova et al., 1993).

Set (4 results from a total of 6)

Identifier: R-HSA-5336183
Species: Homo sapiens
Compartment: extracellular region
Paper describes 'full-length' prosaposin but methods indicate a 'C-teminal fragment' so the actual fragment represented by this full-length prosaposin is unclear.
Identifier: R-HSA-9770781
Species: Homo sapiens
Compartment: nucleoplasm
Identifier: R-HSA-8862963
Species: Homo sapiens
Compartment: lysosomal lumen
Identifier: R-HSA-8862989
Species: Homo sapiens
Compartment: extracellular region

Complex (4 results from a total of 16)

Identifier: R-HSA-9770791
Species: Homo sapiens
Compartment: nucleoplasm
Identifier: R-HSA-5336181
Species: Homo sapiens
Compartment: plasma membrane
Identifier: R-HSA-9842117
Species: Homo sapiens
Compartment: lysosomal lumen
Identifier: R-HSA-9840482
Species: Homo sapiens
Compartment: lysosomal lumen

Interactor (1 results from a total of 1)

Identifier: Q9NZJ7
Species: Homo sapiens
Primary external reference: UniProt: Q9NZJ7

DNA Sequence (2 results from a total of 2)

Identifier: R-HSA-5683891
Species: Homo sapiens
Compartment: nucleoplasm
Primary external reference: ENSEMBL: SFTPA1: ENSEMBL:ENSG00000122852
Identifier: R-HSA-5683880
Species: Homo sapiens
Compartment: nucleoplasm
Primary external reference: ENSEMBL: SFTPA2: ENSEMBL:ENSG00000185303

Icon (2 results from a total of 2)

Species: Homo sapiens
Curator: Bruce May
Designer: Cristoffer Sevilla
SFTPs icon
Generic representation of a Pulmonary surfactant-associated protein family member
Species: Homo sapiens
Curator: Bijay Jassal
Designer: Cristoffer Sevilla
SFTPA2 icon
Pulmonary surfactant-associated protein A2
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