Dolichol-phosphate mannose serves as a donor of mannosyl residues on the lumenal side of the endoplasmic reticulum . Lack of Dol-P-Man results in defective surface expression of GPI-anchored proteins. Dol-P-Man is synthesized from GDP-mannose and dolichol-phosphate on the cytosolic side of the ER by the enzyme dolichyl-phosphate mannosyltransferase. Human DPM1 lacks a carboxy-terminal transmembrane domain and signal sequence and is regulated by DPM2.
CDGIE; dolichol monophosphate mannose synthase; Dolichol-phosphate mannose synthase subunit 1; Dolichol-phosphate mannosyltransferase subunit 1; Dolichyl-phosphate beta-D-mannosyltransferase subunit 1; dolichyl-phosphate mannosyltransferase polypeptide 1; dolichyl-phosphate mannosyltransferase polypeptide 1 catalytic subunit; dolichyl-phosphate mannosyltransferase polypeptide 1, catalytic subunit; DPM synthase complex, catalytic subunit; DPM synthase subunit 1; DPM1; Mannose-P-dolichol synthase subunit 1; MPD synthase subunit 1; MPDS