Glucosidase II is an acidic phosphoprotein enzyme located in the endoplasmic reticulum. The enzyme is responsible for N-linked glycan processing, the mechanism by which proteins undergo proper folding and quality control. The PRKCSH gene encodes the non-catalytic beta subunit of the glucosidase II enzyme. This beta subunit is essential for glucosidase II activity and carries an endoplasmic reticulum luminal retention signal.
Mutations in the PRKCSH gene have been associated with Polycystic Liver Disease 1 (PCLD1), a disorder characterized by multiple fluid filled cysts throughout the liver.