Disorder of dolichol metabolism

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.