2.4.1.131: GDP-Man:Man3GlcNAc2-PP-dolichol alpha-1,2-mannosyltransferase
This is an abbreviated version!
For detailed information about GDP-Man:Man3GlcNAc2-PP-dolichol alpha-1,2-mannosyltransferase, go to the full flat file.
Word Map on EC 2.4.1.131
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2.4.1.131
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lipid-linked
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n-glycosylation
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n-linked
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glycosyltransferase
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dolichol-linked
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asparagine-linked
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man5glcnac2-pp-dolichol
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alpha1,2-linked
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single-subunit
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oligosaccharyltransferase
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man5glcnac2-pp-dol
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microcephaly
- 2.4.1.131
-
lipid-linked
-
n-glycosylation
-
n-linked
- glycosyltransferase
-
dolichol-linked
-
asparagine-linked
-
man5glcnac2-pp-dolichol
-
alpha1,2-linked
-
single-subunit
-
oligosaccharyltransferase
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man5glcnac2-pp-dol
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microcephaly
Reaction
2 GDP-alpha-D-mannose + = 2 GDP +
Synonyms
Alg11, Alg2 mannosyltransferase, At2G40190, galactomannan deficiency protein 3, GDP-Man:Man3GlcNAc2-PP-dolichol-alpha1,2-mannosyltransferase, GDP-mannose-oligosaccharide-lipid mannosyltransferase, gmd3, guanosine diphosphomannose-oligosaccharide-lipid mannosyltransferase, hALG11, LEW3, mannosyltransferase, guanosine diphosphomannose-oligosaccharide-lipid, oligosaccharide-lipid mannosyltransferase
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General Information
General Information on EC 2.4.1.131 - GDP-Man:Man3GlcNAc2-PP-dolichol alpha-1,2-mannosyltransferase
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malfunction
a deletion of the ALG11 gene leads to poor growth and temperature-sensitive lethality, disruption of the ALG11 locus results in Underglycosylation of proteins
malfunction
a patient with muscular hypotonia, convulsions, developmental retardation, dysmorphic signs and death in infancy, a deficiency of GDP-Man:Man3GlcNAc2-PP-dolichol mannosyltransferase, the human ortholog of Alg11 from yeast, is identified as the molecular cause. The enzymatic malfunction leads to impairment in the elongation of lipid-linked oligosaccharides at the outer leaflet of the endoplasmic reticulum, resulting in CDG-Ip, a congenital disorders of glycosylation. Mutation p.L86S in the endoplasmic reticulum mannosyltransferase hALG11 leads to accumulation of Man3GlcNAc2-PP-dolichol and Man4GlcNAc2-PP-dolichol in the index CDG-Ip patient
malfunction
compared with wild-type cells, DELTAalg11 grows poorly and osmotic stabilization by KCl only slightly improved growth. Deletion of ALG11 causes a temperature-sensitive lethality between 32°C and 36°C
malfunction
gmd3 mutant cannot grow at 37°C. Construction of a plasmid pREP1+/-ALG11HA for the expression of Saccharomyces cerevisiae ALG11 in Schizosaccharomyces pombe and introducing it into gmd3 mutant. The gmd3 mutant, carrying pREP+/-ALG11HA, grows at 37°C, whereas the gmd3 mutant, carrying vector pREP1 alone, does not. The defect in acid phosphatase glycosylation of gmd3 mutant is also suppressed by Saccharomyces cerevisiae ALG11. The gmd3+/alg11+ gene is a functional homologue of the Saccharomyces cerevisiae ALG11 gene
malfunction
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mutant lew3, which has a defect in alpha-1,2-mannosyltransferase is deficient in N-glycosylation