Rabbit anti-Human PCSK9 Polyclonal Antibody

Uniprot Id

Q8NBP7

Target Species

Human

Target Name

PCSK9

Target Full Name

Proprotein convertase subtilisin/kexin type 9

Target Function

Crucial player in the regulation of plasma cholesterol homeostasis. Binds to low-density lipid receptor family members: low density lipoprotein receptor (LDLR), very low density lipoprotein receptor (VLDLR), apolipoprotein E receptor (LRP1/APOER) and apolipoprotein receptor 2 (LRP8/APOER2), and promotes their degradation in intracellular acidic compartments. Acts via a non-proteolytic mechanism to enhance the degradation of the hepatic LDLR through a clathrin LDLRAP1/ARH-mediated pathway. May prevent the recycling of LDLR from endosomes to the cell surface or direct it to lysosomes for degradation. Can induce ubiquitination of LDLR leading to its subsequent degradation. Inhibits intracellular degradation of APOB via the autophagosome/lysosome pathway in a LDLR-independent manner. Involved in the disposal of non-acetylated intermediates of BACE1 in the early secretory pathway. Inhibits epithelial Na(+) channel (ENaC)-mediated Na(+) absorption by reducing ENaC surface expression primarily by increasing its proteasomal degradation. Regulates neuronal apoptosis via modulation of LRP8/APOER2 levels and related anti-apoptotic signaling pathways.

Target Involvement

Hypercholesterolemia, autosomal dominant, 3 (HCHOLA3)

Target Subcellular Location

Cytoplasm. Secreted. Endosome. Lysosome. Cell surface. Endoplasmic reticulum. Golgi apparatus. Note=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.

Target Protein Families

Peptidase S8 family

Target Tissue Specificity

Expressed in neuro-epithelioma, colon carcinoma, hepatic and pancreatic cell lines, and in Schwann cells.

Target Research Area

Cardiovascular

Target Synonyms

Convertase subtilisin/kexin type 9 preproprotein; FH3; HCHOLA3; Hypercholesterolemia autosomal dominant 3; LDLCQ1; NARC 1; NARC-1; NARC1; Neural apoptosis regulated convertase 1; Neural apoptosis-regulated convertase 1; PC 9; PC9; PCSK 9; PCSK9; PCSK9_HUMAN; Proprotein convertase 9; Proprotein convertase PC9; Proprotein convertase subtilisin/kexin type 9; PSEC0052; Subtilisin/kexin like protease PC9; Subtilisin/kexin-like protease PC9

Target 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.