The enzyme encoded by this gene catalyzes the first mannosylation step in the biosynthesis of lipid-linked oligosaccharides. This gene is mutated in congenital disorder of glycosylation type Ik. [provided by RefSeq, Dec 2008].
Rind, Schmeiser, Thiel, Absmanner, Lübbehusen, Hocks, Apeshiotis, Wilichowski, Lehle, Körner: "A severe human metabolic disease caused by deficiency of the endoplasmatic mannosyltransferase hALG11 leads to congenital disorder of glycosylation-Ip." in: Human molecular genetics, Vol. 19, Issue 8, pp. 1413-24, (2010) (PubMed).