Table 1.
Genes involved in CoQ biosynthesis and associated with clinical renal pathology
| Human Gene Name | Protein Function | Drosophila Ortholog | Conservation Score | Representative Nephropathy |
|---|---|---|---|---|
| PDSS1 (COQ1 subunit 1) | Catalytic subunit of COQ1, synthesis of CoQ polyisoprene tail | Qless | 9 | None identified |
| PDSS2 (COQ1 subunit 2) | Regulatory subunit of COQ1 | Pdss2 (CG10585) | 10 | NS |
| COQ2 | Transferase, links parahydroxybenzoate redox-active head precursor to polyisoprene tail | Coq2 (CG9613) | 10 | FSGS |
| COQ3 | Methylase, modification of CoQ redox-active head | Coq3 (CG9249) | 10 | None identified |
| COQ4 | Unknown function (regulatory?) | Coq4 (CG32174) | 10 | None identified |
| COQ5 | Methylase, modification of CoQ redox-active head | Coq5 (CG2453) | 9 | None identified |
| COQ6 | Hydroxylase, modification of CoQ redox-active head | Coq6 (CG7277) | 10 | SRNS |
| COQ7 | Hydroxylase, modification of CoQ redox-active head | Coq7 (CG14437) | 8 | None identified |
| COQ8 (ADCK4) | Kinase (regulatory?) | Coq8/Adck4 (CG32649) | 9 | SRNS |
| COQ9 | Unknown function (regulatory?) | Coq9 (CG30493) | 10 | Renal tubulopathy |