Catalytic subunit of glucosidase II that cleaves sequentially the 2 innermost alpha-1,3-linked glucose residues from the Glc(2)Man(9)GlcNAc(2) oligosaccharide precursor of immature glycoproteins . Required for PKD1/Polycystin-1 and PKD2/Polycystin-2 maturation and localization to the cell surface and cilia .
Subcellular locations: Endoplasmic reticulum, Golgi apparatus, Melanosome
Identified by mass spectrometry in melanosome fractions from stage I to stage IV.
Detected in placenta . Isoform 1 and isoform 2 are expressed in the kidney and liver .