Any genetic disorder the prevents adequate synthesis of the lipid dolichol. In N-glycosylation, the oligosaccharide is built upon dolichol before it is transferred to N-glycoproteins. Activated sugars Dol-P-mannose and Dol-P-glucose act as sugar donors for several types of glycosylation occurring in the ER. Thus, disorders of dolichol metabolism negatively affect glycosylation in the ER and impact multiple pathways.
disorders of dolichol metabolism