PCSK9; FH3; HCHOLA3; LDLCQ1; NARC1; PC9; FH3; NARC-1; HCHOLA3; PC9; Proprotein convertase 9; Subtilisin/kexin-like protease PC9; Hypercholesterolemia; autosomal dominant 3; Neural apoptosis-regulated convertase 1;

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Immunogen affinity purified.

Lyophilized from 5 mg BSA, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.05 mg Thimerosal and 0.05 mg NaN3.
Appearance: Lyophilized powder

At -20 degree C
Shelf Life: 12 months

Small volumes of anti-PCSK9 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Polyclonal Antibody to detect PCSK9 in human samples.

Background: Proprotein convertase subtilisin/kexin type 9 (PCSK9), is an enzyme which in humans is encoded by the PCSK9 gene. This gene encodes a proprotein convertase belonging to the proteinase K subfamily of the secretory subtilase family. This protein plays a major regulatory role in cholesterol homeostasis. PCSK9 binds to the epidermal growth factor-like repeat A (EGF-A) domain of the low-density lipoprotein receptor (LDLR), inducing LDLR degradation. PCSK9 may also have a role in the differentiation of cortical neurons. Mutations in this gene have been associated with a rare form of autosomal dominant familial hypercholesterolemia (HCHOLA3).

Antibodies & Supporting Tools; Primary Antibodies (A-Z)

Western Blot (WB), Immunohistochemistry (IHC)-Paraffin

WB, IHC-P

20,827 Da

proprotein convertase subtilisin/kexin type 9

proprotein convertase subtilisin/kexin type 9

Proprotein convertase subtilisin/kexin type 9

NARC1; NARC-1; PC9??[Similar Products]

PCSK9_HUMAN

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]

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: Protease; Secreted, signal peptide; EC 3.4.21.-; Cell development/differentiation; Secreted

Chromosomal Location of Human Ortholog: 1p32.3

Cellular Component: Golgi apparatus; extracellular space; extrinsic to external side of plasma membrane; cell surface; rough endoplasmic reticulum; perinuclear region of cytoplasm; lysosome; endoplasmic reticulum; cytoplasm; late endosome; early endosome; plasma membrane; ER to Golgi transport vesicle

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; positive regulation of neuron apoptosis; phospholipid metabolic process; 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

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