Rat Prosaposin ELISA Kit | MyBioSource MBS721231 product information

product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Rat Prosaposin ELISA Kit

catalog :

MBS721231

quantity :

48-Strip-Wells

price :

470 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS721231

products type :

ELISA Kit

products full name :

Rat Prosaposin ELISA Kit

products short name :

Prosaposin

other names :

PSAP; Prosaposin; prosaposin; proactivator polypeptide; sphingolipid activator protein-1; prosaposin; Proactivator polypeptideCleaved into the following 5 chains:Saposin-AAlternative name(s):Protein A

products gene name :

PSAP

other gene names :

PSAP; PSAP; GLBA; SAP1; GLBA; SAP1; CSAct; SAP-1; SAP-2

uniprot entry name :

SAP_HUMAN

reactivity :

Rat

storage stability :

Store all reagents at 2-8 degree C.

other info1 :

Assay Type: Competitive

other info2 :

Highest Standards: 1000pg/mL. Concentration of Standards: Concentration of Standards: 0,50,100,250,500,1000pg/mL

products categories :

Signal Transduction

ncbi gi num :

48145609

ncbi acc num :

CAG33027.1

uniprot acc num :

P07602

ncbi mol weight :

58,484 Da

ncbi pathways :

Class A/1 (Rhodopsin-like Receptors) Pathway (106357); GPCR Ligand Binding Pathway (161020); Glycosphingolipid Metabolism Pathway (530751); Hemostasis Pathway (106028); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolism Pathway (477135); Metabolism Of Lipids And Lipoproteins Pathway (160976); Peptide Ligand-binding Receptors Pathway (106358); Platelet Activation, Signaling And Aggregation Pathway (106034)

ncbi summary :

This gene encodes a highly conserved glycoprotein which is a precursor for 4 cleavage products: 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, Tay-Sachs disease, and metachromatic leukodystrophy. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008]

uniprot summary :

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: nucleoplasm; Golgi apparatus; lysosomal lumen; extracellular space; mitochondrion; intracellular membrane-bound organelle; lysosomal membrane; extracellular region; integral to membrane; nucleolus. Molecular Function: protein binding; enzyme activator activity; lipid binding. Biological Process: positive regulation of catalytic activity; platelet activation; sphingolipid metabolic process; regulation of lipid metabolic process; platelet degranulation; regulation of MAPKKK cascade; glycosphingolipid metabolic process; lipid transport; blood coagulation. Disease: Gaucher Disease, Atypical, Due To Saposin C Deficiency; Metachromatic Leukodystrophy Due To Saposin B Deficiency; Krabbe Disease, Atypical, Due To Saposin A Deficiency; Combined Saposin Deficiency

size1 :

48-Strip-Wells

price1 :

470 USD

size2 :

96-Strip-Wells

price2 :

675

more info or order :

MyBioSource product webpage