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Entry
N00663                      Network                                

Name
ALG2 deficiency in N-glycan precursor biosynthesis
Definition
P-Dol -- ALG7 >> (ALG13+ALG14) >> ALG1 >> ALG2*
  Expanded
C00110 -- 1798 >> (79868+199857) >> 56052 >> 85365v1
Class
nt06015 N-Glycan biosynthesis
Type
Variant
Pathway
hsa00510  N-Glycan biosynthesis
Disease
H00118  Congenital disorders of glycosylation type I
Gene
1798  ALG7; dolichyl-phosphate N-acetylglucosaminephosphotransferase 1
79868  ALG13; UDP-N-acetylglucosaminyltransferase subunit
199857  ALG14; UDP-N-acetylglucosaminyltransferase subunit
56052  ALG1; chitobiosyldiphosphodolichol beta-mannosyltransferase
85365  ALG2; alpha-1,3/1,6-mannosyltransferase
Variant
85365v1  ALG2 deficiency
Metabolite
C00110  Dolichyl phosphate
Reference
  Authors
Thiel C, Schwarz M, Peng J, Grzmil M, Hasilik M, Braulke T, Kohlschutter A, von Figura K, Lehle L, Korner C.
  Title
A new type of congenital disorders of glycosylation (CDG-Ii) provides new insights into the early steps of dolichol-linked oligosaccharide biosynthesis.
  Journal
J Biol Chem 278:22498-505 (2003)
DOI:10.1074/jbc.M302850200
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