Anti-GYS2

The protein encoded by this gene, liver glycogen synthase, catalyzes the rate-limiting step in the synthesis of glycogen - the transfer of a glucose molecule from UDP-glucose to a terminal branch of the glycogen molecule. Mutations in this gene cause glycogen storage disease type 0 (GSD-0) - a rare type of early childhood fasting hypoglycemia with decreased liver glycogen content. Protein function: Transfers the glycosyl residue from UDP-Glc to the non- reducing end of alpha-1,4-glucan. [The UniProt Consortium]