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Defects in the O‐mannosyl glycan of α‐dystroglycan (α‐DG) are associated with α‐dystroglycanopathy, a group of congenital muscular dystrophies. While α‐DG has many O‐mannosylation sites, only the specific positions can be modified with the functional O‐mannosyl glycan, namely, core M3‐type glycan. POMGNT2 is a glycosyltransferase which adds β1,4‐linked GlcNAc to the O‐mannose (Man) residue to acquire...