Table 1.

Yeast and human genes involved in CoQ biosynthesis

Yeast	Human	Function	Notes
COQ1	COQ1-PDSS1	synthesis of polyprenyl-diphosphate	homotetramer in yeast and heterotetramer in humans
	COQ1-PDSS2
COQ2	COQ2	4HB prenyl-transferase
COQ3	COQ3	O-methyltransferase
COQ4	COQ4	organization of the multienzyme complex	possible role in the maturation of other COQ proteins
COQ5	COQ5	C-methyltransferase
COQ6	COQ6	monooxygenase involved in C5-hydroxylation
COQ7	COQ7	hydroxylase involved in C6-hydroxylation
COQ8	COQ8-ADCK3	regulatory kinase	partially redundant function hypothesized, but precise individual roles are still unclear
	COQ8-ADCK4
COQ9	COQ9	chaperone for COQ7?
COQ10	COQ10a	CoQ chaperone, not directly involved in biosynthesis
	COQ10b