Interaction of the leucine-rich repeats of polycystin-1 with extracellular matrix proteins: possible role in cell proliferation

AN Malhas, RA Abuknesha… - Journal of the American …, 2002 - journals.lww.com
Polycystin-1, the product of the PKD1 gene, is a membrane-bound multidomain protein with
a unique structure and a molecular weight of≈ 460 kD. The purpose of this study is to
investigate the binding of the cystein-flanked leucine-rich repeats (LRR) of polycystin-1 to
extracellular matrix (ECM) components. These interactions may play a role in normal renal
development as well as the pathogenesis of autosomal-dominant polycystic kidney disease
(ADPKD). In vitro assays were used to assess the binding of a fusion protein containing the …