ELISA/assay

Human Prosaposin ELISA Kit

MBS029414

48-Strip-Wells

435 USD

ELISA Kit

Human Prosaposin ELISA Kit

Prosaposin

PSAP; Proactivator polypeptide; proactivator polypeptide; sphingolipid activator protein-1; prosaposin; Protein ASaposin-B-ValSaposin-B; Alternative name(s):; Cerebroside sulfate activator; CSAct; Dispersin; Sphingolipid activator protein 1; SAP-1; Sulfatide/GM1 activatorSaposin-C; Alternative name(s):; A1 activator; Co-beta-glucosidase; Glucosylceramidase activator; Sphingolipid activator protein 2; SAP-2Saposin-D; Alternative name(s):; Component C; Protein C

PSAP

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

SAP_HUMAN

Human

No significant cross-reactivity or interference between Human PSAP and analogues was observed.

Store all reagents at 2-8 degree C

Samples: Serum, Plasma, Tissue Homogenate, Feces, Urine and Body Fluids. Assay Type: Sandwich. Detection Range: 0.25 ng/ml - 8 ng/ml. Sensitivity: 0.1 ng/ml.

Intended Uses: This Quantitative Sandwich ELISA kit is only for in vitro research use only, not for drug, household, therapeutic or diagnostic applications! It is intended to be determinated PSAP concentrations in Human serum, plasma and other body fluids. Using Purified Human PSAP antibody to coat Microelisa Stripplate wells to make solid-phase antibody, then add PSAP and PSAP antibody which has been labeled with HRP to wells, then the reactants become antibody-antigen-antibody-enzyme complex, after washing completely, add TMB substrate solution, TMB substrate becomes blue color under HRP enzyme-catalyzed, reaction is terminated by the addition of a sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450 nm. The concentration of PSAP in the samples is then determined by comparing the O.D. of the samples to the standard curve. Intra-assay Precision: Intra-assay CV (%) is less than 15%. Inter-assay Precision: Inter-assay CV (%) is less than 15%. [CV(%) = SD/mean x100]

Background/Introduction: This Quantitative Sandwich ELISA kit is only for in vitro research use only, not for drug, household, therapeutic or diagnostic applications! It is intended to be determinated PSAP concentrations in Human serum, plasma and other body fluids. Using Purified Human PSAP antibody to coat Microelisa Stripplate wells to make solid-phase antibody, then add PSAP and PSAP antibody which has been labeled with HRP to wells, then the reactants become antibody-antigen-antibody-enzyme complex, after washing completely, add TMB substrate solution, TMB substrate becomes blue color under HRP enzyme-catalyzed, reaction is terminated by the addition of a sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450 nm. The concentration of PSAP in the samples is then determined by comparing the O.D. of the samples to the standard curve.

48145609

CAG33027.1

P07602

58,113 Da

Glycosphingolipid Metabolism Pathway (530751); Hemostasis Pathway (106028); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolism Pathway (477135); Metabolism Of Lipids And Lipoproteins Pathway (160976); Platelet Activation, Signaling And Aggregation Pathway (106034); Platelet Degranulation Pathway (106050); Response To Elevated Platelet Cytosolic Ca2+ Pathway (106048); Sphingolipid Metabolism Pathway (119543)

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]

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; nucleolus; integral to membrane. Molecular Function: protein binding; enzyme activator activity; lipid binding. Biological Process: positive regulation of catalytic activity; platelet activation; platelet degranulation; sphingolipid metabolic process; regulation of lipid metabolic process; regulation of MAPKKK cascade; glycosphingolipid metabolic process; blood coagulation; lipid transport. Disease: Gaucher Disease, Atypical, Due To Saposin C Deficiency; Metachromatic Leukodystrophy Due To Saposin B Deficiency; Combined Saposin Deficiency; Krabbe Disease, Atypical, Due To Saposin A Deficiency

48-Strip-Wells

435 USD

96-Strip-Wells

600