免疫原
Synthesized peptide derived from human PCSK9
特异性
This antibody detects endogenous levels of Human PCSK9
来源
Polyclonal, Rabbit,IgG
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
WB 1:500-2000;IHC-p 1:50-300
纯化工艺（Immunogen）
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
背景（Background）
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.,
功能
urogenital system development, kidney development, liver development, regulation of receptor recycling, negative regulation of receptor recycling, regulation of receptor internalization, positive regulation of receptor internalization,proteolysis, neutral lipid metabolic process, acylglycerol metabolic process, triglyceride metabolic process, phospholipid metabolic process, glycerol ether metabolic process, induction of apoptosis, vacuolar transport, lysosomal transport,steroid metabolic process, cholesterol metabolic process, macromolecule catabolic process, cellular response to starvation, response to endogenous stimulus, response to hormone stimulus, regulation of catabolic process,response to extracellular stimulus, response to organic substance, regulation of receptor activity, positive regulation of macromolecule metabolic process, negative regulation of macromolecule metabolic pro
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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