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Comment
. 2000 Jan;105(2):131-2.
doi: 10.1172/JCI9157.

Congenital disorders of glycosylation caused by defects in mannose addition during N-linked oligosaccharide assembly

P Orlean  1
Affiliations
Comment

Congenital disorders of glycosylation caused by defects in mannose addition during N-linked oligosaccharide assembly

P Orlean. J Clin Invest. 2000 Jan.
No abstract available

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Figures

Figure 1
Figure 1
Assembly of the dolichol-linked precursor in N-glycosylation and steps affected in type I CDG. The Man5GlcNAc2-PP-Dol intermediate is synthesized on the cytoplasmic side of the ER membrane by the successive transfer of GlcNAc and mannose from UDP-GlcNAc and GDP-Man. The Dol-PP-heptasaccharide is then translocated across the ER membrane. Four more mannoses and then 3 glucoses are added from Dol-P-Man and Dol-P-glucose, after which the oligosaccharide is transferred to certain asparagines in protein by oligosaccharyltransferase (OT-ase). Shown are the steps in the formation of GDP-Man and Dol-P-Man and the other glycosylation pathways in which Dol-P-Man can serve as donor. The steps blocked by the known type I CDGs and the genes affected are indicated. F-6-P, fructose-6-P; M, mannose; G, glucose; GN, glucosamine; PI, phosphatidylinositol.
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References

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