Post-translational maturation of dystroglycan is necessary for pikachurin binding and ribbon synaptic localization.

ABSTRACT: Pikachurin, the most recently identified ligand of dystroglycan, plays a crucial role in the formation of the photoreceptor ribbon synapse. It is known that glycosylation of dystroglycan is necessary for its ligand binding activity, and hypoglycosylation is associated with a group of muscular dystrophies that often involve eye abnormalities. Because little is known about the interaction between pikachurin and dystroglycan and its impact on molecular pathogenesis, here we characterize the interaction using deletion constructs and mouse models of muscular dystrophies with glycosylation defects (Large(myd) and POMGnT1-deficient mice). Pikachurin-dystroglycan binding is calcium-dependent and relatively less sensitive to inhibition by heparin and high NaCl concentration, as compared with other

SUBMITTER: Kanagawa M 

PROVIDER: S-EPMC2951195 | biostudies-literature | 2010 Oct

REPOSITORIES: biostudies-literature