ELISA/assay

Mouse Prosaposin ELISA Kit

MBS723297

48-Strip-Wells

470美元

酶联免疫吸附试剂盒

Mouse Prosaposin ELISA Kit

Prosaposin

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

PSAP

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

SAP_HUMAN

小鼠

This assay has high sensitivity and excellent specificity for detection of PSAP. No significant cross-reactivity or interference between PSAP and analogues was observed. NOTE: Limited by current skills and knowledge, it is impossible for us to complete the cross-reactivity detection between PSAP and all the analogues, therefore, cross reaction may still exist in some cases.

Store all reagents at 2-8 degree C.

Samples: Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate. Assay Type: Competitive. Sensitivity: 1.0 pg/mL.

Intended Uses: This PSAP ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Mouse PSAP. This ELISA kit for research use only, not for therapeutic or diagnostic applications!

信号转导

Principle of the assay: PSAP ELISA kit applies the competitive enzyme immunoassay technique utilizing a monoclonal anti-PSAP antibody and an PSAP-HRP conjugate. The assay sample and buffer are incubated together with PSAP-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the PSAP concentration since PSAP from samples and PSAP-HRP conjugate compete for the anti-PSAP antibody binding site. Since the number of sites is limited, as more sites are occupied by PSAP from the sample, fewer sites are left to bind PSAP-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The PSAP concentration in each sample is interpolated from this standard curve.

48145609

CAG33027.1

P07602

58,484 Da

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)

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; integral to membrane; extracellular region; nucleolus. Molecular Function: protein binding; enzyme activator activity; lipid binding. Biological Process: positive regulation of catalytic activity; platelet activation; regulation of lipid metabolic process; sphingolipid 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

48-Strip-Wells

470美元

96-Strip-Wells

675