Abstract |
In the majority of congenital disorders of glycosylation, the assembly of the glycan precursor GlcNAc(2)Man(9)Glc(3) on the polyprenol carrier dolichyl-pyrophosphate is compromised. Because N-linked glycosylation is essential to life, most types of congenital disorders of glycosylation represent partial losses of enzymatic activity. Consequently, increased availability of substrates along the glycosylation pathway can be beneficial to increase product formation by the compromised enzymes. Recently, we showed that increased dolichol availability and improved N-linked glycosylation can be achieved by inhibition of squalene biosynthesis. This review summarizes the current knowledge on the biosynthesis of dolichol-linked glycans with respect to deficiencies in N-linked glycosylation. Additionally, perspectives on therapeutic treatments targeting dolichol and dolichol-linked glycan biosynthesis are examined.
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Authors | Michael Welti |
Journal | Glycoconjugate journal
(Glycoconj J)
Vol. 30
Issue 1
Pg. 51-6
(Jan 2013)
ISSN: 1573-4986 [Electronic] United States |
PMID | 22717794
(Publication Type: Journal Article, Review)
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Chemical References |
- Dolichol Phosphates
- Dolichols
- Polysaccharides
- dolichol pyrophosphate
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Topics |
- Carbohydrate Sequence
(genetics)
- Congenital Disorders of Glycosylation
(metabolism, pathology)
- Dolichol Phosphates
(metabolism)
- Dolichols
(genetics, metabolism)
- Glycosylation
- Humans
- Polysaccharides
(genetics, metabolism)
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