Human PSAP Recombinant Protein (RPPB4300)
- SKU:
- RPPB4300
- Product Type:
- Recombinant Protein
- Species:
- Human
- Uniprot:
- P07602
Description
| Product Name: | Human PSAP Recombinant Protein |
| Product Code: | RPPB4300 |
| Size: | 10µg |
| Species: | Human |
| Target: | PSAP |
| Synonyms: | Prosaposin, Proactivator polypeptide, PSAP, GLBA, SAP1. |
| Source: | HEK293 Cells |
| Physical Appearance: | Filtered White lyophilized (freeze-dried) powder. |
| Formulation: | Filtered (0.4�m) and lyophilized from 0.5mg/ml in phosphate buffer, pH 7.4. |
| Solubility: | It is recommended to add deionized water to prepare a working stock solution of approximately 0.5mg/ml and let the lyophilized pellet dissolve completely. PSAP is not sterile! Please filter the product by an appropriate sterile filter before using it in the cell culture. |
| Stability: | Store lyophilized protein at -20°C. Aliquot the product after reconstitution to avoid repeated freezing/thawing cycles. Reconstituted protein can be stored at 4°C for a limited period of time; it does not show any change after two weeks at 4°C. |
| Purity: | Greater than 95.0% as determined by SDS-PAGE. |
| Amino Acid Sequence: | ASGPVLGLKE CTRGSAVWCQ NVKTASDCGA VKHCLQTVWN KPTVKSLPCD ICKDVVTAAG DMLKDNATEE EILVYLEKTC DWLPKPNMSA SCKEIVDSYL PVILDIIKGE MSRPGEVCSA LNLCESLQKH LAELNHQKQL ESNKIPELDM TEVVAPFMAN IPLLLYPQDG PRSKPQPKDN GDVCQDCIQM VTDIQTAVRT NSTFVQALVE HVKEECDRLG PGMADICKNY ISQYSEIAIQ MMMHMQPKEI CALVGFCDEV KEMPMQTLVP AKVASKNVIP ALELVEPIKK HEVPAKSDVY CEVCEFLVKE VTKLIDNNKT EKEILDAFDK MCSKLPKSLS EECQEVVDTY GSSILSILLE EVSPELVCSM LHLCSGTRLP ALTVHVTQPK DGGFCEVCKK LVGYLDRNLE KNSTKQEILA ALEKGCSFLP DPYQKQCDQF VAEYEPVLIE ILVEVMDPSF VCLKIGACPS AHKPLLGTEK CIWGPSYWCQ NTETAAQCNA VEHCKRHVWN KLHHHHHH |
Prosaposin (PSAP) is a precursor of 4 lysosomal saposin proteins (saposin A, B, C and D). Saposins are heat-stable glycoproteins which facilitate the catabolism of glycosphingolipids with short oligosaccharide groups. PSAP stimulates neurite outgrowth and increases choline acetyltransferase activity. Prosaposin exists both as a secretory protein and as an integral membrane protein and has neurotrophic activities. The secreted prosaposin is assumed to have additional functions in human milk, cerebrospinal fluid and seminal plasma. PSAP gene mutations are linked with Gaucher disease, Tay-Sachs disease, and metachromatic leukodystrophy.
PSAP Human Recombinant produced in HEK cells is a single, glycosylated, polypeptide chain (a.a 17-524) containing a total of 518 amino acids, having a molecular mass of 57.7kDa (calculated), though it migrates at approximately 65kDa on SDS PAGE, the PSAP is fused to a 2 a.a N-terminal linker, a 2 a.a C-terminal linker and a 6 a.a His tag at C-Terminus.The Human PSAP is purified by proprietary chromatographic techniques.
| UniProt Protein Function: | 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. |
| UniProt Protein Details: | 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: blood coagulation; G-protein signaling, adenylate cyclase inhibiting pathway; glycosphingolipid metabolic process; lipid transport; platelet activation; platelet degranulation; positive regulation of catalytic activity; positive regulation of MAPKKK cascade; regulation of lipid metabolic process; sphingolipid 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 |
| NCBI Summary: | 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 Code: | P07602 |
| NCBI GenInfo Identifier: | 134218 |
| NCBI Gene ID: | 5660 |
| NCBI Accession: | P07602.2 |
| UniProt Secondary Accession: | P07602,P07292, P15793, P78538, P78541, P78546, P78547 P78558, Q53Y86, Q6IBQ6, Q92739, Q92740, |
| UniProt Related Accession: | P07602 |
| Molecular Weight: | 58,484 Da |
| NCBI Full Name: | Prosaposin |
| NCBI Synonym Full Names: | prosaposin |
| NCBI Official Symbol: | PSAP�� |
| NCBI Official Synonym Symbols: | GLBA; SAP1�� |
| NCBI Protein Information: | prosaposin |
| UniProt Protein Name: | Prosaposin |
| UniProt Synonym Protein Names: | Proactivator polypeptide |
| Protein Family: | Prosaposin |
| UniProt Gene Name: | PSAP�� |
| UniProt Entry Name: | SAP_HUMAN |
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