Mannose-P-dolichol utilization defect 1 protein (MPDU1)

Mannose-P-dolichol utilization defect 1 protein (MPDU1) is an endoplasmic reticulum membrane protein essential for the proper utilization of the mannose donor, mannose-P-dolichol, in the biosynthesis of N-linked glycans and glycosylphosphatidylinositol (GPI) anchors[1][5]. MPDU1 is required for the flipping and subsequent usage of dolichol-linked monosaccharides within the ER, critical for forming mature glycan structures in proteins. Mutations in MPDU1 disrupt glycosylation, leading to a multisystemic metabolic disease known as congenital disorder of glycosylation type If (CDG-If), with severe molecular and clinical manifestations[1]. MPDU1 belongs to a family of membrane cargo receptors with seven transmembrane domains, implicated in protein trafficking and metabolite transport[2]. There are no existing approved drugs directly targeting MPDU1, and its deficiency is typically detected by analyzing glycosylation patterns such as serum transferrin isoelectric focusing and accumulation of specific oligosaccharide intermediates[1][5].

Other names

Lec35CDGIFHBEBP2BPASL15PQLC5SLC66A5My008PP3958

Not applicable due to the lack of directly interacting drugs