Exogenous expression of the glycosyltransferase LARGE1 restores α-dystroglycan matriglycan and laminin binding in rhabdomyosarcoma.
Daniel BeltránMary E AndersonNarendra BharathyTeagan P SettelmeyerMatthew N SvalinaZia BajwaJohn F ShernSakir H GultekinMarco A CuellarTakahiro YonekawaCharles KellerKevin P CampbellPublished in: Skeletal muscle (2019)
Collectively, our findings demonstrate that a lack of matriglycan on α-dystroglycan is a common feature in RMS due to the downregulation of LARGE1, and that ectopic expression of LARGE1 can restore matriglycan modifications and the ability of α-dystroglycan to function as an ECM receptor.