Gene name: COQ6

Uniprot entry:

Q9Y2Z9

Protein names:

Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial (EC 1.14.13.-) (Coenzyme Q10 monooxygenase 6)

Protein sequence:

1_MAARL 6_ VSRCG 11_ AVRAA 16_ PHSGP 21_ LVSWR 26_ RWSGA 31_ STDTV 36_ YDVVV 41_ SGGGL 46_ VGAAM 51_ ACALG 56_ YDIHF 61_ HDKKI 66_ LLLEA 71_ GPKKV 76_ LEKLS 81_ ETYSN 86_ RVSSI 91_ SPGSA 96_ TLLSS 101_ FGAWD 106_ HICNM 111_ RYRAF 116_ RRMQV 121_ WDACS 126_ EALIM 131_ FDKDN 136_ LDDMG 141_ YIVEN 146_ DVIMH 151_ ALTKQ 156_ LEAVS 161_ DRVTV 166_ LYRSK 171_ AIRYT 176_ WPCPF 181_ PMADS 186_ SPWVH 191_ ITLGD 196_ GSTFQ 201_ TKLLI 206_ GADGH 211_ NSGVR 216_ QAVGI 221_ QNVSW 226_ NYDQS 231_ AVVAT 236_ LHLSE 241_ ATENN 246_ VAWQR 251_ FLPSG 256_ PIALL 261_ PLSDT 266_ LSSLV 271_ WSTSH 276_ EHAAE 281_ LVSMD 286_ EEKFV 291_ DAVNS 296_ AFWSD 301_ ADHTD 306_ FIDTA 311_ GAMLQ 316_ YAVSL 321_ LKPTK 326_ VSARQ 331_ LPPSV 336_ ARVDA 341_ KSRVL 346_ FPLGL 351_ GHAAE 356_ YVRPR 361_ VALIG 366_ DAAHR 371_ VHPLA 376_ GQGVN 381_ MGFGD 386_ ISSLA 391_ HHLST 396_ AAFNG 401_ KDLGS 406_ VSHLT 411_ GYETE 416_ RQRHN 421_ TALLA 426_ ATDLL 431_ KRLYS 436_ TSASP 441_ LVLLR 446_ TWGLQ 451_ ATNAV 456_ SPLKE 461_QIMAF

Protein annotations

Protein functions:

1: FAD-dependent monooxygenase required for two non-consecutive steps during ubiquinone biosynthesis (PubMed:26260787, PubMed:38425362). Required for the C5-ring hydroxylation during ubiquinone biosynthesis by catalyzing the hydroxylation of 4-hydroxy-3-(all-trans-decaprenyl)benzoic acid to 3,4-dihydroxy-5-(all-trans-decaprenyl)benzoic acid (PubMed:26260787, PubMed:38425362). Also acts downstream of COQ4, for the C1-hydroxylation during ubiquinone biosynthesis by catalyzing the hydroxylation of 2-methoxy-6-(all-trans-decaprenyl)phenol to 2-methoxy-6-(all-trans-decaprenyl)benzene-1,4-diol (PubMed:38425362). The electrons required for the hydroxylation reaction are funneled indirectly to COQ6 from NADPH via a ferredoxin/ferredoxin reductase system composed of FDX2 and FDXR (PubMed:26260787, PubMed:38425362)