Glucosidase II alpha subunit (GANAB)

Glucosidase II alpha subunit (encoded by the GANAB gene) is the catalytic component of a heterodimeric enzyme in the ER responsible for the sequential removal of α1,3-linked glucose residues from N-linked oligosaccharides on glycoproteins. This two-step glucose trimming, accomplished with the β-regulatory subunit, facilitates ER-associated protein folding, quality control, and subsequent transport. Its activity generates a transient protein tag recognized by lectin chaperones like calnexin and calreticulin, promoting proper maturation or facilitating refolding before misfolded proteins are targeted for degradation. Dysregulation or mutation of GANAB is causative of autosomal dominant polycystic kidney and liver disease and may be implicated in cancer and other protein-folding disorders

Other names

GANABGIIαGII alphaNeutral glycosyl hydrolase 31 alpha subunitGlucosidase 2 subunit alpha

Drugs or inhibitors targeting this molecule act by blocking its catalytic active site and preventing hydrolysis of α1,3-linked glucose residues from N-glycans