UbiA prenyltransferase domain-containing protein 1 (UBIAD1)

UbiA prenyltransferase domain-containing protein 1 (UBIAD1) is a membrane-bound enzyme belonging to the UbiA superfamily of prenyltransferases. It catalyzes the transfer of isoprenoid side chains to molecules, mediating the synthesis of vitamin K2 (menaquinone-4) and participating in the coenzyme Q10 biosynthesis pathway, both critical for mitochondrial function and cellular redox balance. UBIAD1 is widely expressed in human tissues and involved in cholesterol and phospholipid homeostasis. Loss-of-function mutations in UBIAD1 cause Schnyder crystalline corneal dystrophy, a rare disorder characterized by progressive lipid and cholesterol accumulation in the cornea. UBIAD1 also acts as a tumor suppressor in the prostate and urinary tract, regulating cell proliferation through MAPK and cholesterol-associated pathways. The enzyme can be inhibited by lipophilic statins and interacts with steroid metabolism, mitochondrial function, and cardiovascular health.

Other names

Transitional epithelial response protein 1TERE1Transitional epithelia response proteinSCCD geneUbiA prenyltransferase domain containing 1UbiA prenyltransferase domain-containing protein 1

Statins inhibit UBIAD1 directly, influencing prenyltransferase activity and downstream vitamin K2/coenzyme Q10 production. Geranylgeraniol relieves UBIAD1-dependent inhibition of HMG-CoA reductase degradation, affecting sterol regulatory processes.