Antigen: purified human recombinant PCSK9 · Clone designation: 15A6 · Host: mouse · Isotype: IgG₁ · Application(s): PCSK9 ligand blotting (nondenaturing conditions) and WB · Proprotein convertase subtilisin kexin 9 (PCSK9) is a member of the subtilisin serine protease family with an important role in lipoprotein metabolism.¹ Gain-of-function mutations in the PCSK9 gene are associated with autosomal dominant hypercholesterolemia which is characterized by an increase in low-density lipoprotein (LDL) cholesterol levels.² PCSK9 overexpression in wild-type mice doubles the plasma total cholesterol, possibly through accelerated LDL receptor degredation.^3,1 PCSK9 mRNA is detected in various tissues such as liver, kidney, lung, spleen, jejunum, ileum, colon, and muscle with the highest expression in the liver.⁴ Human PCSK9 precursor is 692 amino acids in length with an estimated molecular weight of 74 kDa. This proprotein is self-cleaved to form a mature protein at around 63 kDa in the Golgi body.⁵

1 Maxwell, K.N., Fisher, E.A., and Breslow, J.L. Overexpression of PCSK9 accelerates the degradation of the LDLR in a post-endoplasmic reticulum compartment. Proc Natl Acad Sci USA 102(6) 2069-2074 (2005).

2 Abifadel, M., Varret, M., Rabès, J., et al. Mutations in PCSK9 cause autosomal dominant hypercholesterolemia. Nat Genet 34(2) 154-156 (2003).

3 Maxwell, K.N., and Breslow, J.L. Adenoviral-mediated expression of Pcsk9 in mice results in a low-density lipoprotein receptor knockout phenotype. Proc Natl Acad Sci USA 101(18) 7100-7105 (2004).

4 Seidah, N.G., Benjannet, S., Wickham, L., et al. The secretory proprotein convertase neural apoptosis-regulated convertase 1 (NARC-1): Liver regeneration and neuronal differentiation. Proc Natl Acad Sci USA 100(3) 928-933 (2003).

5 Maxwell, K.N., and Breslow, J.L. Proprotein convertase subtilisin kexin 9: The third locus implicated in autosomal dominant hypercholesterolemia. Curr Opin Lipidol 16 167-172 (2005).

Maxwell, K.N., and Breslow, J.L. Proprotein convertase subtilisin kexin 9: The third locus implicated in autosomal dominant hypercholesterolemia. Curr Opin Lipidol 16 167-172 (2005).

Abifadel, M., Varret, M., Rabès, J., et al. Mutations in PCSK9 cause autosomal dominant hypercholesterolemia. Nat Genet 34(2) 154-156 (2003).

Maxwell, K.N., Fisher, E.A., and Breslow, J.L. Overexpression of PCSK9 accelerates the degradation of the LDLR in a post-endoplasmic reticulum compartment. Proc Natl Acad Sci USA 102(6) 2069-2074 (2005).

Lagace, T.A., Curtis, D.E., Garuti, R., et al. Secreted PCSK9 decreases the number of LDL receptors in hepatocytes and in livers of parabiotic mice. J Clin Invest 116(11) 2995-3005 (2006).

Seidah, N.G., Benjannet, S., Wickham, L., et al. The secretory proprotein convertase neural apoptosis-regulated convertase 1 (NARC-1): Liver regeneration and neuronal differentiation. Proc Natl Acad Sci USA 100(3) 928-933 (2003).

Maxwell, K.N., and Breslow, J.L. Adenoviral-mediated expression of Pcsk9 in mice results in a low-density lipoprotein receptor knockout phenotype. Proc Natl Acad Sci USA 101(18) 7100-7105 (2004).

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