Article
NARC-1/PCSK9 and its natural mutants: zymogen cleavage and effects on the low density lipoprotein (LDL) receptor and LDL cholesterol.
The Journal of biological chemistry - 19 Nov 2004
Benjannet Suzanne, Rhainds David, Essalmani Rachid, Mayne Janice, Wickham Louise, Jin Weijun, Asselin Marie-Claude, Hamelin Josée, Varret Mathilde, Allard Delphine, Trillard Mélanie, Abifadel Marianne, Tebon Angie, Attie Alan D, Rader Daniel J, Boileau Catherine, Brissette Louise, Chrétien Michel, Prat Annik, Seidah Nabil G
Abstract excerpt
The discovery of autosomal dominant hypercholesterolemic patients with mutations in the PCSK9 gene, encoding the proprotein convertase NARC-1, resulting in the missense mutations suggested a role in low density lipoprotein (LDL) metabolism. We show that the endoplasmic reticulum-localized proNARC-1 to NARC-1 zymogen conversion is Ca2+-independent and that within the zymogen autocatalytic processing site SSVFAQ...
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