The o-mannosylation pathway: Glycosyltransferases and proteins implicated in congenital muscular dystrophy

Abstract

Several forms of congenital muscular dystrophy, referred to as dystroglycanopathies, result from defects in the proteinO-mannosylation biosynthetic pathway. In this minireview, I discuss 12 proteins involved in the pathway and how they play a role in the building of glycan structures (most notably on the protein ?-dystroglycan) that allow for binding to multiple proteins of the extracellular matrix. © 2013 by The American Society for Biochemistry and Molecular Biology, Inc.