| Modification Notes |
HUGT2 is also known as UGCGL2 (UDP-glucose ceramide glucosyltransferase-like 2) or UGT2 (UDP-glucose: glycoprotein glucosyltransferase 2) and is a 1,516 amino acid protein that is expressed abundantly in kidney, pancreas, heart and skeletal muscle. HUGT2 is localized to the lumen of the ER (endoplasmic reticulum) and to the ER-Golgi intermediate compartment which regulates transportation between the ER and the Golgi. HUGT2 is a quality control protein that targets unfolded glycoproteins for glucosylation in a calcium-dependent manner, but may use manganese is the absence of calcium. HUGT2 targets glycans by attaching single glucose residues to glycans with amino acid sequences which are not correctly folded, an enzymatic process that is exhibited during carbohydrate processing. Patients with type 2 diabetes express altered HUGT2 proteins in their beta cells, suggesting that these patients may also have defects in their ER glycoprotein quality control abilities. |