Dolichol-P-Man dependent alpha (1–3) mannosyltransferase; involved in synthesis of dolichol-linked oligosaccharide donor for N-linked glycosylation of proteins; G353A missense mutation in human ortholog ALG3 implicated in carbohydrate deficient glycoprotein syndrome type IV, which is characterized by microcephaly, severe epilepsy, minimal psychomotor development and partial deficiency of sialic acids in serum glycoproteins; wild-type human ALG3 can complement yeast alg3 mutant. The gene discussed is ALG3; the disease is ALG3-congenital disorder of glycosylation.