This gene 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.