免疫原
Synthesized peptide derived from human PCSK9
特异性
This antibody detects endogenous levels of PCSK9 at Human, Mouse,Rat
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.84% sodium azide.
纯化工艺（Immunogen）
The antibody was affinity-purified from mouse ascites by affinity-chromatography using specific immunogen.
背景（Background）
This gene encodes a member of the subtilisin-like proprotein convertase family, which includes proteases that process protein and peptide precursors trafficking through regulated or constitutive branches of the secretory pathway. The encoded protein undergoes an autocatalytic processing event with its prosegment in the ER and is constitutively secreted as an inactive protease into the extracellular matrix and trans-Golgi network. It is expressed in liver, intestine and kidney tissues and escorts specific receptors for lysosomal degradation. It plays a role in cholesterol and fatty acid metabolism. Mutations in this gene have been associated with autosomal dominant familial hypercholesterolemia. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Feb 2014],
功能
cofactor:Calcium.,disease:Defects in PCSK9 are the cause of familial hypercholesterolemia 3 (FH3) [MIM:603776]. FH3 inheritance is autosomal dominant.,enzyme regulation:Inhibited by EGTA.,function:May be implicated in the differentiation of cortical neurons and may play a role in cholesterol homeostasis.,PTM:The soluble zymogen undergoes autocatalytic intramolecular processing in the endoplasmic reticulum, resulting in the cleavage of its propeptide that remains associated with the secreted enzyme.,similarity:Belongs to the peptidase S8 family.,similarity:Contains 1 peptidase S8 domain.,subunit:The precursor protein but not the mature protein may form multimers.,tissue specificity:Expressed in neuro-epithelioma, colon carcinoma, hepatic and pancreatic cell lines, and in Schwann cells.,
基因名称（Gene Name）
PCSK9 NARC1 PSEC0052
其他名称
Proprotein convertase subtilisin/kexin type 9 (EC 3.4.21.-) (Neural apoptosis-regulated convertase 1) (NARC-1) (Proprotein convertase 9) (PC9) (Subtilisin/kexin-like protease PC9)
Fields
>>Cholesterol metabolism
细胞定位
Cytoplasm. Secreted. Endosome. Lysosome. Cell surface. Endoplasmic reticulum. Golgi apparatus. Autocatalytic cleavage is required to transport it from the endoplasmic reticulum to the Golgi apparatus and for the secretion of the mature protein. Localizes to the endoplasmic reticulum in the absence of LDLR and colocalizes to the cell surface and to the endosomes/lysosomes in the presence of LDLR. The sorting to the cell surface and endosomes is required in order to fully promote LDLR degradation.
组织表达
Expressed in neuro-epithelioma, colon carcinoma, hepatic and pancreatic cell lines, and in Schwann cells.
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