N-acetylglucosamine-1-phosphate transferase


N-acetylglucosamine-1-phosphate transferase is a transferase enzyme.

Function

It is made up of two alpha, two betas, and two gammas subunits. GNPTAB produces the alpha and beta subunits, GNPTG produces the gamma subunit. GlcNAc-1-phosphotransferase functions to prepare newly made enzymes for lysosome transportation. Lysosomes, a part of an animal cell, helps break down large molecules into smaller ones that can be reused. GlcNAc-1-phosphotransferase catalyzes the N-linked glycosylation of asparagine residues with a molecule called mannose-6-phosphate. M6P acts as an indicator of whether a hydrolase should be transported to the lysosome or not. Once a hydrolase indicates an M6P, it can be transported to a lysosome. Surprisingly some lysosomal enzymes are only tagged at a rate of 5% or lower.

Clinical significance

It is associated with the following conditions:
In melanocytic cells, GNPTG gene expression may be regulated by MITF.