Rabbit PSAP Polyclonal Antibody | anti-PSAP antibody

PSAP Rabbit Polyclonal

Cat. Num. AAA7603076

• Gene Names: PSAP; GLBA; SAP1
• Reactivity: Human, Mouse, Rat
• Applications: ELISA, Western Blot, Immunohistochemistry
• Purity: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Synonyms: PSAP; Polyclonal Antibody; PSAP Rabbit Polyclonal; anti-PSAP antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Human, Mouse, Rat; other species are not tested. Please decide the specificity by homology.
• Purity/Purification: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Form/Format: Liquid
• Sequence Length: 524

• Applicable Applications for anti-PSAP antibody: ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: WB: 1:500 - 1:1000; IHC: 1:50 - 1:200
• Immunogen: Prosaposin
• Preparation and Storage: PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human liver cancer using MBS7603076 (PSAP antibody) at dilution of 1:50)

Western Blot (WB)

(HepG2 cells were subjected to SDS PAGE followed by western blot with MBS7603076 (PSAP Antibody) at dilution of 1:600)

Related Product Information for anti-PSAP antibody

Saposin-A and saposin-C stimulate the hydrolysis of glucosylceramide by beta-glucosylceramidase(EC 3.2.1.45) and galactosylceramide by beta-galactosylceramidase(EC 3.2.1.46). Saposin-C apparently acts by combining with the enzyme and acidic lipid to form an activated complex, rather than by solubilizing the substrate. Saposin-B stimulates the hydrolysis of galacto-cerebroside sulfate by arylsulfatase A(EC 3.1.6.8), GM1 gangliosides by beta-galactosidase(EC 3.2.1.23) and globotriaosylceramide by alpha-galactosidase A(EC 3.2.1.22). Saposin-B forms a solubilizing complex with the substrates of the sphingolipid hydrolases. Saposin-D is a specific sphingomyelin phosphodiesterase activator(EC 3.1.4.12). Prosaposin: Behaves as a myelinotrophic and neurotrophic factor, these effects are mediated by its G-protein-coupled receptors, GPR37 and GPR37L1, undergoing ligand-mediated internalization followed by ERK phosphorylation signaling. Saposins are specific low-molecular mass non-enzymic proteins, they participate in the lysosomal degradation of sphingolipids, which takes place by the sequential action of specific hydrolases.

NCBI and Uniprot Product Information

• NCBI GI #: 48145609
• NCBI GeneID: 5660
• UniProt Accession #: P07602; P07292; P15793; P78538; P78541; P78546; P78547; P78558; Q53Y86; Q6IBQ6; Q92739; Q92740
• Molecular Weight: 60 kDa
• NCBI Official Full Name: PSAP
• NCBI Official Synonym Full Names: prosaposin
• NCBI Official Symbol: PSAP
• NCBI Official Synonym Symbols: GLBA; SAP1
• NCBI Protein Information: prosaposin
• UniProt Protein Name: Prosaposin
• Protein Family: Prosaposin
• UniProt Gene Name: PSAP
• UniProt Synonym Gene Names: GLBA; SAP1; CSAct; SAP-1; SAP-2
• UniProt Entry Name: SAP_HUMAN

NCBI Description

This gene encodes a highly conserved preproprotein that is proteolytically processed to generate four main cleavage products including saposins A, B, C, and D. Each domain of the precursor protein is approximately 80 amino acid residues long with nearly identical placement of cysteine residues and glycosylation sites. Saposins A-D localize primarily to the lysosomal compartment where they facilitate the catabolism of glycosphingolipids with short oligosaccharide groups. The precursor protein exists both as a secretory protein and as an integral membrane protein and has neurotrophic activities. Mutations in this gene have been associated with Gaucher disease and metachromatic leukodystrophy. Alternative splicing results in multiple transcript variants, at least one of which encodes an isoform that is proteolytically processed. [provided by RefSeq, Feb 2016]

Uniprot Description

PSAP: The lysosomal degradation of sphingolipids takes place by the sequential action of specific hydrolases. Some of these enzymes require specific low-molecular mass, non-enzymic proteins: the sphingolipids activator proteins (coproteins). Defects in PSAP are the cause of combined saposin deficiency (CSAPD); also known as prosaposin deficiency. CSAPD is due to absence of all saposins, leading to a fatal storage disorder with hepatosplenomegaly and severe neurological involvement. Defects in PSAP saposin-B region are the cause of leukodystrophy metachromatic due to saposin-B deficiency (MLD- SAPB). MLD-SAPB is an atypical form of metachromatic leukodystrophy. It is characterized by tissue accumulation of cerebroside-3-sulfate, demyelination, periventricular white matter abnormalities, peripheral neuropathy. Additional neurological features include dysarthria, ataxic gait, psychomotr regression, seizures, cognitive decline and spastic quadriparesis. Defects in PSAP saposin-C region are the cause of atypical Gaucher disease (AGD). Affected individuals have marked glucosylceramide accumulation in the spleen without having a deficiency of glucosylceramide-beta glucosidase characteristic of classic Gaucher disease, a lysosomal storage disorder. Defects in PSAP saposin-A region are the cause of atypical Krabbe disease (AKRD). AKRD is a disorder of galactosylceramide metabolism. AKRD features include progressive encephalopathy and abnormal myelination in the cerebral white matter resembling Krabbe disease. Defects in PSAP saposin-D region are found in a variant of Tay-Sachs disease (GM2-gangliosidosis). 3 isoforms of the human protein are produced by alternative splicing.

Chromosomal Location of Human Ortholog: 10q21-q22

Cellular Component: cytoplasm; extracellular region; extracellular space; integral to membrane; lysosomal lumen; lysosomal membrane; mitochondrion

Molecular Function: enzyme activator activity; G-protein-coupled receptor binding; lipid binding; protein binding

Biological Process: G-protein signaling, adenylate cyclase inhibiting pathway; glycosphingolipid metabolic process; lipid transport; platelet degranulation; positive regulation of catalytic activity; positive regulation of MAPKKK cascade; regulation of autophagy; regulation of lipid metabolic process

Disease: Combined Saposin Deficiency; Gaucher Disease, Atypical, Due To Saposin C Deficiency; Krabbe Disease, Atypical, Due To Saposin A Deficiency; Metachromatic Leukodystrophy Due To Saposin B Deficiency