Alpha-1,6-mannosyl-glycoprotein 2-beta-N-acetylglucosaminyltransferase

Definition:

Plays an essential role in protein N-glycosylation. Catalyzes the transfer of N-acetylglucosamine (GlcNAc) onto the free terminal mannose moiety in the core structure of the nascent N-linked glycan chain, giving rise to the second branch in complex glycans.

References:

[1]. V Cormier-Daire, et al. Congenital disorders of glycosylation IIa cause growth retardation, mental retardation, and facial dysmorphism. J Med Genet. 2000 Nov;37(11):875-7. [Content Brief]

[2]. Renuka Kadirvelraj, et al. Human N-acetylglucosaminyltransferase II substrate recognition uses a modular architecture that includes a convergent exosite. Proc Natl Acad Sci U S A. 2018 May 1;115(18):4637-4642. [Content Brief]

[3]. J Tan, et al. Mutations in the MGAT2 gene controlling complex N-glycan synthesis cause carbohydrate-deficient glycoprotein syndrome type II, an autosomal recessive disease with defective brain development. Am J Hum Genet. 1996 Oct;59(4):810-7. [Content Brief]

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