Reactome | Addition of a GlcNAc on the alpha 1,4 branch by MGAT2

Addition of a GlcNAc on the alpha 1,4 branch by MGAT2

Stable Identifier

R-HSA-975829

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi membrane, Golgi lumen

Locations in the PathwayBrowser

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Metabolism of proteins (Homo sapiens)

- Post-translational protein modification (Homo sapiens)
  - Asparagine N-linked glycosylation (Homo sapiens)
    - Transport to the Golgi and subsequent modification (Homo sapiens)
      - N-glycan antennae elongation in the medial/trans-Golgi (Homo sapiens)
        
        Reactions specific to the complex N-glycan synthesis pathway (Homo sapiens)
        
        Addition of a GlcNAc on the alpha 1,4 branch by MGAT2 (Homo sapiens)

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Addition of a GlcNAc on the alpha 1,4 branch by MGAT2

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The addition of a GlcNAc on the alpha-1,6 mannose on the alpha-1,4 branch is required for the synthesis of complex N-glycans (Tan et al. 1995). Defects in this gene are associated with Congenital Disorder of Glycosylation type IIa (Tan et al. 1996, Wang et al. 2002).

Literature References

PubMed ID	Title	Journal	Year
8808595	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 Tan, J, Dunn, J, Jaeken, J, Schachter, H	Am J Hum Genet	1996
12417412	Mice with a homozygous deletion of the Mgat2 gene encoding UDP-N-acetylglucosamine:alpha-6-D-mannoside beta1,2-N-acetylglucosaminyltransferase II: a model for congenital disorder of glycosylation type IIa Wang, Y, Schachter, H, Marth, JD	Biochim Biophys Acta	2002
7635144	The human UDP-N-acetylglucosamine: alpha-6-D-mannoside-beta-1,2- N-acetylglucosaminyltransferase II gene (MGAT2). Cloning of genomic DNA, localization to chromosome 14q21, expression in insect cells and purification of the recombinant protein Tan, J, D'Agostaro, AF, Bendiak, B, Reck, F, Sarkar, M, Squire, JA, Leong, P, Schachter, H	Eur J Biochem	1995