The dystrophin glycoprotein complex, which connects the cell membrane to the basement
membrane, is essential for a variety of biological events, including maintenance of muscle …

Protein O-linked mannose β1, 2-N-acetylglucosaminyltransferase 1 (POMGnT1) catalyzes
the transfer of GlcNAc to O-mannose of glycoproteins. Mutations in the POMGnT1 gene …

Protein O-mannose β1, 2-N-acetyglucosaminyltransferase 1 (POMGnT1) is an enzyme
involved in the synthesis of O-mannosyl glycans. Mutations of POMGnT1 in humans result in …

Dystroglycanopathies are a subset of congenital muscular dystrophies wherein α-
dystroglycan (α-DG) is hypoglycosylated. α-DG is an extensively O-glycosylated …

α-Dystroglycan is a component of the dystrophin-glycoprotein-complex, which is the major
mechanism of attachment between the cytoskeleton and the extracellular matrix. Muscle …

Muscle–eye–brain disease (MEB), an autosomal recessive disorder, is characterized by
congenital muscular dystrophy, brain malformation, and ocular abnormalities. Previously, we …

Mammalian cells produce many glycoproteins, ie, proteins with covalently attached sugar
chains. Recent advances in glycobiology have revealed the importance of sugar chains as …

The main extracellular matrix binding component of the dystrophin-glycoprotein complex, α-
dystroglycan (α-DG), which was originally isolated from rabbit skeletal muscle, is an …

α-Dystroglycan (α-DG) is a membrane-associated glycoprotein that interacts with several
extracellular matrix proteins, including laminin and agrin. Aberrant glycosylation of α-DG …

Disruption of the O-mannosylation pathway involved in functional glycosylation of α-
dystroglycan gives rise to congenital muscular dystrophies. Protein O-linked mannose β-1, 4 …