Anti-Proprotein Convertase Subtilisin/Kexin Type 9 Antibody, Non-Fucosylated (BioBet-1590ZP) (CAT#: BioBet-1590ZP) Datasheet

PCSK9

PCSK9; proprotein convertase subtilisin/kexin type 9; FH3; PC9; NARC1; LDLCQ1; NARC-1; HCHOLA3; subtilisin/kexin-like protease PC9; neural apoptosis regulated convertase 1; convertase subtilisin/kexin type 9 preproprotein;

proprotein convertase subtilisin/kexin type 9

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]

Golgi apparatus, Extracellular region or secreted, Endosome, Lysosome, Endoplasmic reticulum

Diseases associated with PCSK9 include Hypercholesterolemia, Familial, 3 and Familial Hypercholesterolemia.

Its related pathways are Neuroscience and Lipoprotein metabolism.

Play a key role in regulating plasma cholesterol homeostasis. With members of the low density lipid receptor family: low density lipoprotein receptor (LDLR), very low density lipoprotein receptor (VLDLR), apolipoprotein E receptor (LRP1/APOER) and apolipoprotein receptor 2 ( The combination of LRP8/APOER2) promotes its degradation in the acidic compartment of the cell. Through a non-proteolytic mechanism, through the clathrin LDLRAP1/arh-mediated pathway, the degradation of liver LDLR is enhanced. It may prevent LDLR from circulating from endosomes to the cell surface or direct it to lysosome degradation. Can induce ubiquitination of LDLR, leading to its degradation. The autophagosome/lysosome pathway inhibits APOB degradation in cells in a ldlr-independent manner. Involved in processing the non-acetylated intermediates of BACE1 in the early secretory pathway. Inhibition of epithelial Na(+) channel (ENaC)-mediated Na(+) absorption, mainly by increasing the proteasome degradation of ENaC to reduce its surface expression. Regulate neuronal apoptosis by regulating LRP8/APOER2 levels and related anti-apoptotic signaling pathways.

Cell Biology and Cellular Response antibody; Developmental Biology antibody; Metabolism antibody; Signaling Transduction antibody

Cleavage by furin and PCSK5 generates a truncated inactive protein that is unable to induce LDLR degradation. Undergoes autocatalytic cleavage in the endoplasmic reticulum to release the propeptide from the N-terminus and the cleavage of the propeptide is strictly required for its maturation and activation. The cleaved propeptide however remains associated with the catalytic domain through non-covalent interactions, preventing potential substrates from accessing its active site. As a result, it is secreted from cells as a propeptide-containing, enzymatically inactive protein. Phosphorylation protects the propeptide against proteolysis.

Protein Based Therapies
Monoclonal antibody (mAb)

IgG

BioBet-1590ZP

Humanized

Human

The humanized mAb recognizes Proprotein convertase subtilisin/kexin type 9 (PCSK9). It can be used to treat cardiovascular dyslipidemia.

Cardiovascular Dyslipidemia

Cardiovascular Dyslipidemia