Antigen: synthetic peptide from mouse PCSK9 amino acids 152-163 (VFAQSIPWNLER) · Host: rabbit · Application(s): WB and ICC; other applications not tested · Proprotein convertase subtilisin kexin 9 (PCSK9) is a member of the subtilisin serine protease family with an important role in lipoprotein metabolism.¹ Mutation in the PCSK9 gene is 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 acceleration of the degradation of the LDL receptor.^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 acid 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.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., 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).

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. 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).

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).

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

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