Asparagine Linked Glycosylation Protein 8 (ALG8)

ALG8 encodes a member of the ALG6/ALG8 glucosyltransferase family. The encoded protein catalyzes the addition of the second glucose residue to the lipid-linked oligosaccharide precursor for N-linked glycosylation of proteins. Mutations in this gene have been associated with congenital disorder of glycosylation type Ih (CDG-Ih). Alternatively spliced transcript variants encoding different isoforms have been identified.ALG8 protein contains 532 amino acids. It shares 38% amino acid identity with yeast ALG8.By database analysis, Oriol et al. (2002) identified ALG8. They determined that the 526-amino acid protein has 12 transmembrane domains and an endoplasmic reticulum retention signal (KTKKQ).