Asparagine Linked Glycosylation Protein 2 (ALG2)

ALG2 encodes a member of the glycosyltransferase 1 family. The encoded protein acts as an alpha 1,3 mannosyltransferase, mannosylating Man(2)GlcNAc(2)-dolichol diphosphate and Man(1)GlcNAc(2)-dolichol diphosphate to form Man(3)GlcNAc(2)-dolichol diphosphate. Defects in this gene have been associated with congenital disorder of glycosylation type Ih (CDG-Ii).By searching databases for homologs of yeast Alg2, Thiel et al. (2003) identified human ALG2. The predicted 416-amino acid ALG2 protein shares 37% identity with yeast Alg2.Thiel et al. (2003) showed that human ALG2 acts as an alpha-1,3-mannosyltransferase. The product of this reaction is further elongated by an alpha-1,6-mannosyltransferase.