POMGNT2

JCGG-STR032527		JCGG-STR023421

POMGNT2, also known as GTDC2 or AGO61, is a protein O-linked mannose β1,4-N-acetylglucosaminyltransferase 2. POMGNT2 transfers GlcNAc to the C4 position of O-mannose on a-dystroglycan. Mutations in POMGNT2 cause lack of O-mannosyl trisaccharide (GalNAc-GlcNAc-Man-Ser/Thr), which is a precursor of laminin-binding glycans to regulate cellular ashesion to the basal membrane.
O-mannose congenital muscular dystrophy dystroglycanopathy α-dystroglycan

Glc	Man	Gal	GlcNAc	GalNAc	Fuc	Xyl	Neu5Ac	Neu5Gc	GlcA	IdoA	GlcN