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货号: YP-Ab-02777
促销价:

产品介绍

反应种属
Human;Rat;Mouse;
应用范围
WB;IHC;IF;ELISA
抗体类型
多克隆抗体
基因名称(Gene Name)
PSAP
蛋白名称
Proactivator polypeptide
分子量(DA)
58kD
免疫原
The antiserum was produced against synthesized peptide derived from human PSAP. AA range:307-356
特异性
Saposin Polyclonal Antibody detects endogenous levels of Saposin protein.
组成
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
来源
Polyclonal, Rabbit,IgG
稀释比例
WB: 1/500 - 1/2000. IHC: 1/100 - 1/300. ELISA: 1/20000.. IF 1:50-200
纯化工艺
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
浓度
1 mg/ml
储存
-20°C/1 year
其他名称
PSAP; GLBA; SAP1; Proactivator polypeptide
背景
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],
功能
alternative products:Additional isoforms seem to exist,disease:Defects in PSAP are the cause of combined saposin deficiency (CSAPD) [MIM:611721]; 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.,disease:Defects in PSAP saposin-A region are the cause of atypical Krabbe disease (AKRD) [MIM:611722]. AKRD is a disorder of galactosylceramide metabolism. AKRD features include progressive encephalopathy and abnormal myelination in the cerebral white matter resembling Krabbe disease.,disease:Defects in PSAP saposin-B region are the cause of a variant of metachromatic leukodystrophy (MLD) [MIM:249900].,disease:Defects in PSAP saposin-C region are the cause of atypical Gaucher disease (AGD) [MIM:610539]. Affected individuals have marked glucosylceramide accumulation in the

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