ALG11

ALG11

A gene on chromosome 13q14.2 that encodes GDP-Man:Man3GlcNAc2-PP-dolichol-alpha1,2-mannosyltransferase, an enzyme localised to the cytosolic side of the endoplasmic reticulum that catalyses the transfer of the 4th and 5th mannose residue from GDP-mannose (GDP-Man) to Man3GlcNAc2-PP-dolichol and Man4GlcNAc2-PP-dolichol, resulting in the production of Man5GlcNAc2-PP-dolichol.
Molecular pathology
ALG11 is mutated in congenital disorder of glycosylation type Ip (CDGIp).