Youngah Jo, Jason S Hamilton ... Russell A DeBose-Boyd
UBIAD1 mediates a unique geranylgeranyl pyrophosphate-sensing mechanism that when disrupted, inhibits degradation of HMG CoA reductase and triggers overproduction of corneal cholesterol that characterizes the eye disease Schnyder Corneal Dystrophy.
Marc M Schumacher, Rania Elsabrouty ... Russell A DeBose-Boyd
The inhibition of sterol-accelerated degradation of HMG CoA reductase by the vitamin K2 synthetic enzyme UBIAD1 may contribute to the accumulation of cholesterol that is associated with Schnyder corneal dystrophy.
Youngah Jo, Steven S Kim ... Russell A DeBose-Boyd
Embryonic lethality associated with deficiency of UBIAD1, which synthesizes a vitamin K2 subtype, results from aberrant ER-associated degradation of the cholesterol biosynthetic enzyme HMG CoA reductase.
Rania Elsabrouty, Youngah Jo ... Russell A DeBose-Boyd
Type 1 polyisoprenoid diphosphate phosphatase (PDP1) contributes to interconversion of isoprenols and isoprenylpyrophosphates, balancing the sterol and nonsterol branches of the mevalonate pathway by regulating ERAD of HMG CoA reductase and ER-to-Golgi transport of UBIAD1.
