UDP-N-acetylglucosamine–lysosomal-enzyme N-acetylglucosaminephosphotransferase

UDP-N-ac·e·tyl·glu·co·sa·mine–ly·so·so·mal-en·zyme N-ac·e·tyl·glu·co·sa·mine·phos·pho·trans·fer·ase (as″ə-tēl″gloo-kōsґə-mēn li″so-soґməl enґzīm as″ə-tēl″gloo-kōs″ə-mēn-fos″fo-transґfər-ās) [EC 2.7.8.17] an enzyme of the transferase class that catalyzes a step in the synthesis of the mannose 6-phosphate recognition markers necessary on most lysosomal enzymes for internalization of the enzymes into lysosomes. It is a hexameric protein comprising two alpha, two beta, and two gamma subunits; the subunits are encoded by two separate genes. Deficiency of the enzyme results in mucolipidosis type II or III, with the specific type related to the subunit or subunits affected as well as to the level of enzyme activity remaining. Called also N-acetylglucosaminylphosphotransferase.