Background:
This gene encodes a component of the coenzyme Q biosynthesis pathway. Coenzyme Q; an essential component of the electron transport chain; shuttles electrons between complexes I or II to complex III of the mitochondrial transport chain. This protein appears to play a structural role in stabilizing a complex that contains most of the coenzyme Q biosynthesis enzymes. Mutations in this gene are associated with mitochondrial disorders linked to coenzyme Q deficiency. Alternative splicing results in multiple transcript variants. [provided by RefSeq; Apr 215]