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Gene Review

MGAT2  -  mannosyl (alpha-1,6-)-glycoprotein beta-1...

Homo sapiens

Synonyms: Alpha-1,6-mannosyl-glycoprotein 2-beta-N-acetylglucosaminyltransferase, Beta-1,2-N-acetylglucosaminyltransferase II, CDG2A, CDGS2, GLCNACTII, ...
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Disease relevance of MGAT2

  • 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 [1].
  • The discovery of the MGAT2 gene will facilitate studies to determine the functional role of MGAT2 in fat absorption in the intestine and to determine whether blocking MGAT activity in enterocytes is a feasible approach to inhibit fat absorption and treat obesity [2].
  • Recombinant human UDP-GlcNAc: alpha-Man-(1-->6)R beta-(1-->2)-N-acetylglucosaminyltransferase II (EC, GlcNAc-T II) was produced in the Sf9 insect cell/baculovirus expression system as a fusion protein with a (His)6 tag and partially purified by affinity chromatography on a metal chelating column [3].

High impact information on MGAT2

  • Two unrelated CDGS type II patients are shown to have point mutations (one patient having Ser-->Phe and the other having His-->Arg) in the catalytic domain of the gene MGAT2, encoding UDP-GlcNAc:alpha-6-D-mannoside beta-1,2-N- acetylglucosaminyltransferase II (GnT II), an enzyme essential for biosynthesis of complex Asn-linked glycans [1].
  • MGAT2, a monoacylglycerol acyltransferase expressed in the small intestine [2].
  • Expression of the MGAT2 cDNA in either insect or mammalian cells markedly increased MGAT activity in cell membranes [2].
  • In humans, the MGAT2 gene is highly expressed in the small intestine, liver, stomach, kidney, colon, and white adipose tissue; in mice, it is expressed predominantly in the small intestine [2].
  • Thus novel glycosylation pathways have been uncovered in two such knockouts, namely alpha-mannosidase II null mice and UDP-N-acetylglucosamine: alpha 6-D-mannoside beta 1,2-N-acetylglucosaminyltransferase II null mice [4].

Biological context of MGAT2


Anatomical context of MGAT2


Associations of MGAT2 with chemical compounds


Other interactions of MGAT2


  1. 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) [Pubmed]
  2. MGAT2, a monoacylglycerol acyltransferase expressed in the small intestine. Yen, C.L., Farese, R.V. J. Biol. Chem. (2003) [Pubmed]
  3. Synthesis of pentasaccharide analogues of the N-glycan substrates of N-acetylglucosaminyltransferases III, IV and V using tetrasaccharide precursors and recombinant beta-(1-->2)-N-acetylglucosaminyltransferase II. Reck, F., Meinjohanns, E., Tan, J., Grey, A.A., Paulsen, H., Schachter, H. Carbohydr. Res. (1995) [Pubmed]
  4. MS screening strategies: investigating the glycomes of knockout and myodystrophic mice and leukodystrophic human brains. Sutton-Smith, M., Morris, H.R., Grewal, P.K., Hewitt, J.E., Bittner, R.E., Goldin, E., Schiffmann, R., Dell, A. Biochem. Soc. Symp. (2002) [Pubmed]
  5. 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, A.F., Bendiak, B., Reck, F., Sarkar, M., Squire, J.A., Leong, P., Schachter, H. Eur. J. Biochem. (1995) [Pubmed]
  6. Transcriptional regulation of the human UDP-GlcNAc:alpha-6-D-mannoside beta-1-2-N-acetylglucosaminyltransferase II gene (MGAT2) which controls complex N-glycan synthesis. Chen, S.H., Zhou, S., Tan, J., Schachter, H. Glycoconj. J. (1998) [Pubmed]
  7. Human intestinal monoacylglycerol acyltransferase: differential features in tissue expression and activity. Lockwood, J.F., Cao, J., Burn, P., Shi, Y. Am. J. Physiol. Endocrinol. Metab. (2003) [Pubmed]
  8. Hypoglycosylation of a brain glycoprotein (beta-trace protein) in CDG syndromes due to phosphomannomutase deficiency and N-acetylglucosaminyl-transferase II deficiency. Pohl, S., Hoffmann, A., Rüdiger, A., Nimtz, M., Jaeken, J., Conradt, H.S. Glycobiology (1997) [Pubmed]
  9. Carbohydrate-deficient glycoprotein syndrome type II. Schachter, H., Jaeken, J. Biochim. Biophys. Acta (1999) [Pubmed]
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