2.4.1.142: chitobiosyldiphosphodolichol beta-mannosyltransferase
This is an abbreviated version!
For detailed information about chitobiosyldiphosphodolichol beta-mannosyltransferase, go to the full flat file.
Word Map on EC 2.4.1.142
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2.4.1.142
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oligosaccharide
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n-glycosylation
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mannosylation
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dolichol-linked
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lipid-linked
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n-linked
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n-glycans
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asparagine-linked
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disease-causing
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glycosylphosphatidylinositol
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glycolipids
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rough
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pharmacology
- 2.4.1.142
- oligosaccharide
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n-glycosylation
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mannosylation
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dolichol-linked
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lipid-linked
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n-linked
- n-glycans
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asparagine-linked
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disease-causing
- glycosylphosphatidylinositol
- glycolipids
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rough
- pharmacology
Reaction
Synonyms
ALG1, ALG1 beta1,4 mannosyltransferase, Alg1 mannosyltransferase, Alg1 protein, Alg1p, beta-1,4 mannosyltransferase, beta1,4-MT, chitobiosyldiphosphodolichol beta-mannosyltransferase, GDP-Man:GlcNAc2-PP-dolichol mannosyltransferase, GDP-mannose-dolichol diphosphochitobiose mannosyltransferase, guanosine diphosphate-mannose:GlcNAc2-PP-dolichol mannosyltransferase-1, guanosine diphosphomannose-dolichol diphosphochitobiose mannosyltransferase, mannosyltransferase I, MT-1, MT-I
ECTree
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Substrates Products
Substrates Products on EC 2.4.1.142 - chitobiosyldiphosphodolichol beta-mannosyltransferase
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REACTION DIAGRAM
GDP-alpha-D-mannose + chitobiosyldiphosphodolichol
GDP + beta-(1->4)-D-mannosylchitobiosyldiphosphodolichol
GDP-alpha-D-mannose + diphosphodolichyl-N-acetyl-D-glucosaminyl-N-acetyl-D-glucosamine
GDP + beta-(1->4)-D-mannosylchitobiosyl diphosphodolichol
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GDP-alpha-D-mannose + N-acetyl-beta-D-glucosaminyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
GDP + beta-D-mannosyl-(1->4)-N-acetyl-beta-D-glucosaminyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
GDP + beta-(1->4)-D-mannosylchitobiosyldiphosphodolichol
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GDP-alpha-D-mannose + chitobiosyldiphosphodolichol
GDP + beta-(1->4)-D-mannosylchitobiosyldiphosphodolichol
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GDP-alpha-D-mannose + chitobiosyldiphosphodolichol
GDP + beta-(1->4)-D-mannosylchitobiosyldiphosphodolichol
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GDP-alpha-D-mannose + chitobiosyldiphosphodolichol
GDP + beta-(1->4)-D-mannosylchitobiosyldiphosphodolichol
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GDP-alpha-D-mannose + N-acetyl-beta-D-glucosaminyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
GDP + beta-D-mannosyl-(1->4)-N-acetyl-beta-D-glucosaminyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
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GDP-alpha-D-mannose + N-acetyl-beta-D-glucosaminyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
GDP + beta-D-mannosyl-(1->4)-N-acetyl-beta-D-glucosaminyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
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GDP-alpha-D-mannose + N-acetyl-beta-D-glucosaminyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
GDP + beta-D-mannosyl-(1->4)-N-acetyl-beta-D-glucosaminyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
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GDP-alpha-D-mannose + N-acetyl-beta-D-glucosaminyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
GDP + beta-D-mannosyl-(1->4)-N-acetyl-beta-D-glucosaminyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
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GDP-alpha-D-mannose + N-acetyl-beta-D-glucosaminyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
GDP + beta-D-mannosyl-(1->4)-N-acetyl-beta-D-glucosaminyl-(1->4)-N-acetyl-alpha-D-glucosaminyl-diphosphodolichol
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patient with congenital disorder of glycosylation is compound heterozygous for three mutations in the ALG1 gene, leading to the amino acid substitutions S150R and D429E on one allele and S258L on the other. The detrimental effect of these mutations on ALG1 protein function is demonstrated in a complementation assay. This novel type of congenital disorder of glycosylation should be referred to as CDG-Ik
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additional information
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the molecular nature of severe multisystemic disorder with a recurrent nonimmune hydrops fetalis is identified as deficiency of GDP-Man:GlcNAc2-PP-dolichol mannosyltransferase caused by a C773T transition
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additional information
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two distinct complexes that contain multiple copies of Alg1 mannosyltransferase are identified. The two Alg1-containing complexes differ from one another in that one complex contains Alg2 and the other contains Alg11. Physical association of the functionally related catalytic enzymes involved in lipid-linked oligosaccharide synthesis provides an additional level of regulation that assures proper construction of the complex oligosaccharides
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additional information
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Alg1 protein transfers a second beta-linkage mannose to the GlcNAc2-PP-Phy (phytanyl-diphosphoryl-alpha-N, N'-diacetylchitobioside) substrate
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additional information
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Alg1 protein transfers a second beta-linkage mannose to the GlcNAc2-PP-Phy (phytanyl-diphosphoryl-alpha-N, N'-diacetylchitobioside) substrate
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additional information
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chemo-enzymatic synthesis of lipid-linked GlcNAc2Man5 oligosaccharides using recombinant Alg1, Alg2 and Alg11 proteins. Comparison to the reaction of dolichyl-diphosphooligosaccharide-protein glycosyltransferase subunit STT3A (EC 2.4.99.18)
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additional information
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chemo-enzymatic synthesis of lipid-linked GlcNAc2Man5 oligosaccharides using recombinant Alg1, Alg2 and Alg11 proteins. Comparison to the reaction of dolichyl-diphosphooligosaccharide-protein glycosyltransferase subunit STT3A (EC 2.4.99.18)
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additional information
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Alg1 protein transfers a second beta-linkage mannose to the GlcNAc2-PP-Phy (phytanyl-diphosphoryl-alpha-N, N'-diacetylchitobioside) substrate
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