PCSK9, Polyclonal Antibody

FH3; PC9; NARC1; LDLCQ1; NARC-1; HCHOLA3

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

Polyclonal

IgG

Rabbit

Affinity Purification

1mg/ml (lot specific)

Store at -20 degree C (regular) or -80 degree C (long term). Avoid freeze / thaw cycles.
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

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.

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.

Polyclonal

Western Blot (WB), Immunohistochemistry (IHC)

WB: 1:500 - 1:1000
IHC: 1:50 - 1:100

Western blot analysis of extracts of various cell lines, using PCSK9 antibody.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.

692

proprotein convertase subtilisin/kexin type 9

proprotein convertase subtilisin/kexin type 9; subtilisin/kexin-like protease PC9; neural apoptosis regulated convertase 1; convertase subtilisin/kexin type 9 preproprotein

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

Chromosomal Location of Human Ortholog: 1p32.3

Cellular Component: Golgi apparatus; extracellular space; cell surface; extrinsic to external side of plasma membrane; rough endoplasmic reticulum; lysosome; perinuclear region of cytoplasm; endoplasmic reticulum; early endosome; cytoplasm; late 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; liver development; proteolysis; cellular response to starvation; neuron differentiation; protein autoprocessing; cholesterol homeostasis; triacylglycerol metabolic process; cellular response to insulin stimulus; neurogenesis; 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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