NP_777596.2
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NCBI GenBank Nucleotide #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
20,827 Da
NCBI Official Full Name
proprotein convertase subtilisin/kexin type 9 preproprotein
NCBI Official Synonym Full Names
proprotein convertase subtilisin/kexin type 9
NCBI Official Synonym Symbols
FH3; PC9; NARC1; LDLCQ1; NARC-1; HCHOLA3 [Similar Products]
NCBI Protein Information
proprotein convertase subtilisin/kexin type 9; subtilisin/kexin-like protease PC9; neural apoptosis regulated convertase 1; convertase subtilisin/kexin type 9 preproprotein
UniProt Protein Name
Proprotein convertase subtilisin/kexin type 9
UniProt Synonym Protein Names
Neural apoptosis-regulated convertase 1; NARC-1; Proprotein convertase 9; PC9; Subtilisin/kexin-like protease PC9
UniProt Synonym Gene Names
UniProt Entry Name
PCSK9_HUMAN
NCBI Summary for PCSK9
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]
UniProt Comments for PCSK9
PCSK9: 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. Defects in PCSK9 are the cause of hypercholesterolemia autosomal dominant type 3 (HCHOLA3). A familial condition characterized by elevated circulating cholesterol contained in either low-density lipoproteins alone or also in very-low-density lipoproteins. Belongs to the peptidase S8 family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 3.4.21.-; Cell development/differentiation; Secreted; Secreted, signal peptide; Protease
Chromosomal Location of Human Ortholog: 1p32.3
Cellular Component: Golgi apparatus; extracellular space; cell surface; rough endoplasmic reticulum; endoplasmic reticulum; lysosome; early endosome; ER to Golgi transport vesicle; extrinsic to external side of plasma membrane; perinuclear region of cytoplasm; late endosome; cytoplasm; plasma membrane
Molecular Function: very-low-density lipoprotein binding; sodium channel inhibitor activity; protein binding; protein self-association; low-density lipoprotein receptor binding; serine-type endopeptidase activity; low-density lipoprotein binding; apolipoprotein binding; apolipoprotein receptor binding
Biological Process: cholesterol metabolic process; lysosomal transport; apoptosis; positive regulation of receptor internalization; lipoprotein metabolic process; regulation of low-density lipoprotein receptor catabolic process; cellular response to starvation; proteolysis; liver development; neuron differentiation; protein autoprocessing; cholesterol homeostasis; triacylglycerol metabolic process; neurogenesis; cellular response to insulin stimulus; phospholipid metabolic process; positive regulation of neuron apoptosis; regulation of neuron apoptosis; negative regulation of receptor recycling; low-density lipoprotein receptor catabolic process; regulation of receptor activity; kidney development
Disease: Hypercholesterolemia, Autosomal Dominant, 3
Research Articles on PCSK9
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Diseases associated with PCSK9 elisa kit
Organs/Tissues associated with PCSK9 elisa kit
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