Protein O-linked-mannose beta-1,4-N-acetylglucosaminyltransferase 2

Definition:

O-linked mannose beta-1,4-N-acetylglucosaminyltransferase that transfers UDP-N-acetyl-D-glucosamine to the 4-position of the mannose to generate N-acetyl-D-glucosamine-beta-1,4-O-D-mannosylprotein. Involved in the biosynthesis of the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1), which is required for binding laminin G-like domain-containing extracellular proteins with high affinity.

References:

[1]. Yukari Endo, et al. Milder forms of muscular dystrophy associated with POMGNT2 mutations. Neurol Genet. 2015 Dec 10;1(4):e33. [Content Brief]

[2]. Takako Yoshida-Moriguchi, et al. SGK196 is a glycosylation-specific O-mannose kinase required for dystroglycan function. Science. 2013 Aug 23;341(6148):896-9. [Content Brief]

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