抗体

Rabbit anti-human prosaposin polyclonal Antibody

MBS713289

0.05毫升

230美元

多克隆

兔

未共轭

人类, 小鼠, 大鼠

免疫印迹, 酶联免疫吸附测定, 免疫组化, 酶免疫法

抗体

Rabbit anti-human prosaposin polyclonal Antibody

[prosaposin]

[prosaposin; PSAP; FLJ00245; GLBA; MGC110993; SAP1]

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

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

SAP_HUMAN

多克隆

IgG

兔

人类, 小鼠, 大鼠

13

抗原亲和纯化

酶联免疫吸附测定(EIA), 免疫印迹(免疫印迹), 免疫组化(免疫组化)

Immunogen: Human PSAP

Storage Buffer: PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20 degree C, Avoid freeze / thaw cycles. Santa Cruz Alternative: Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-27014 / sc-27018 / sc-27012 / sc-32875

11139234

AAG31635.1

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; extracellular space; lysosomal lumen; intracellular membrane-bound organelle; mitochondrion; lysosomal membrane; nucleolus; extracellular region; integral to membrane. Molecular Function: protein binding; enzyme activator activity; lipid binding. Biological Process: positive regulation of catalytic activity; platelet activation; sphingolipid metabolic process; platelet degranulation; 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; Krabbe Disease, Atypical, Due To Saposin A Deficiency; Combined Saposin Deficiency

0.05毫升

230美元

0.15毫升

500