[HTML][HTML] Molecular characterization of proprotein convertase subtilisin/kexin type 9-mediated degradation of the LDLR

Y Wang, Y Huang, HH Hobbs, JC Cohen - Journal of lipid research, 2012 - ASBMB
Proprotein convertase subtilisin/kexin type 9 (PCSK9) is a secreted protein that promotes
degradation of cell surface LDL receptors (LDLRs) in selected cell types. Here we used
genetic and pharmacological inhibitors to define the pathways involved in PCSK9-mediated
LDLR degradation. Inactivating mutations in autosomal recessive hypercholesterolemia
(ARH), an endocytic adaptor, blocked PCSK9-mediated LDLR degradation in lymphocytes
but not in fibroblasts. Thus, ARH is not specifically required for PCSK9-mediated LDLR …