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Analysis of Glycosylation in CDG-Ia Fibroblasts by Fluorophore-assisted Carbohydrate Electrophoresis: IMPLICATIONS FOR EXTRACELLULAR GLUCOSE AND INTRACELLULAR MANNOSE 6-PHOSPHATE
Phosphomannomutase (PMM) deficiency causes congenital disorder of glycosylation (CDG)-Ia, a broad spectrum disorder with developmental and neurological abnormalities. PMM converts mannose 6-phosphate (M6P) to mannose-1-phosphate, a precursor of GDP-mannose used to make Glc(3)Man(9)GlcNAc(2)-P-P-doli...
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Main Authors: | , , |
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Formato: | Artigo |
Idioma: | Inglês |
Publicado em: |
2005
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Assuntos: | |
Acesso em linha: | https://ncbi.nlm.nih.gov/pmc/articles/PMC1282451/ https://ncbi.nlm.nih.gov/pubmed/15708848 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M500510200 |
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