Search Results

Overview

Uniprot IDQ8R1S0
Protein NameUbiquinone biosynthesis monooxygenase COQ6, mitochondrial
Gene NameCoq6
OrganismMus musculus

Kla Sites from experimental identification

Position Flanking peptide
330 RHAVALLKPTKVSAR
72 DIHFHDKKILLLEAG
86 GPKKALEKLSETYSN

Function

FAD-dependent monooxygenase required for two non-consecutive steps during ubiquinone biosynthesis. 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. 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. 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

Protein Sequence

10 MAARIGSMAG 20 LLCVRWWSSA 30 QLAARGGPLV 40 ASQRWAGSSA 50 DTVYDVVVSG 60 GGLVGSAMAC 70 ALGHDIHFHD 80 KKILLLEAGP 90 KKALEKLSET 100 YSNRVSSISP 110 GSTTLLSSFG 120 AWDHICNMRC 130 KAFRRMQVWD 140 SCSEALIMFD 150 RDNLDDMGYI 160 VENDVIMYAL 170 TKQLEAVADR 180 VKVLYESKAV 190 GYSWPGAFSM 200 ADSSPWVHIT 210 LGDGSTLQTK 220 LLIGADGHKS 230 GVRQAAGIQN 240 VSWKYDQSAV 250 VATLHLSEAT 260 ENNVAWQRFL 270 PSGPIALLPL 280 SDTLSSLVWS 290 TSHEHAAELV 300 SMDEEEFVDA 310 INSAFWSDVH 320 HTDFVDSASA 330 MVRHAVALLK 340 PTKVSARQLP 350 PSIAKVDAKS 360 RALFPLGLGH 370 AAEYVRPRVA 380 LIGDAAHRIH 390 PLAGQGVNMG 400 FGDISSLVHH 410 LSTAAFNGKD 420 LGSMSHLTGY 430 ETDRQRHNTA 440 LLAATDLLKR 450 LYSTSATPLV 460 LLRTWGLQAT 470 NAVSPLKEQI MAFASK

Gene Ontology

Classification GO ID Description
Cellular Component GO:0042995 cell projection
Cellular Component GO:0031314 extrinsic component of mitochondrial inner membrane
Cellular Component GO:0005794 Golgi apparatus
Cellular Component GO:0005743 mitochondrial inner membrane
Cellular Component GO:0005739 mitochondrion
Cellular Component GO:0110142 ubiquinone biosynthesis complex
Molecular Function GO:0120538 2-methoxy-6-polyprenolphenol 4-hydroxylase activity
Molecular Function GO:0106364 4-hydroxy-3-all-trans-polyprenylbenzoate oxygenase activity
Molecular Function GO:0071949 FAD binding
Molecular Function GO:0016491 oxidoreductase activity
Molecular Function GO:0016709 oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, NAD(P)H as one donor, and incorporation of one atom of oxygen
Molecular Function GO:0016712 oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen
Biological Process GO:0006744 ubiquinone biosynthetic process

Reference

[1] Zhuo W, Zhang M, Tan J, Gao Y, Wang Y et al.. Lysine lactylation analysis of proteins in the heart of the Kawasaki disease mouse model.. Front Cell Dev Biol 13:1550220. 2025. PMID: 40114965.

[2] Wu D, Tang Y, Li X, Xiong S, Zhang Z et al.. Characterization of protein lactylation in healthy and ischemic mouse hearts.. Front Cardiovasc Med 12:1644886. 2025. PMID: 41089239.