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Details on Person UniProt:Q68FU7 Coq6

Class:IdReferenceGeneProduct:2587580
_chainChangeLogchain:1-476 added on Fri February 6 2015;chain:1-476 removed on Fri May 8 2015;transit peptide:1-42 added on Fri May 8 2015;chain:43-476 added on Fri May 8 2015
_displayNameUniProt:Q68FU7 Coq6
_timestamp2025-08-15 21:13:48
chaintransit peptide:1-42
chain:43-476
checksumCD4D2BC7D7C03880
commentFUNCTION 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.CATALYTIC ACTIVITY 4-hydroxy-3-(all-trans-decaprenyl)benzoate + 2 reduced [2Fe-2S]-[ferredoxin] + O2 + 2 H(+) = 3,4-dihydroxy-5-(all-trans-decaprenyl)benzoate + 2 oxidized [2Fe-2S]-[ferredoxin] + H2OCATALYTIC ACTIVITY 2-methoxy-6-(all-trans-decaprenyl)phenol + 2 reduced [2Fe-2S]-[ferredoxin] + O2 + 2 H(+) = 2-methoxy-6-(all-trans-decaprenyl)benzene-1,4-diol + 2 oxidized [2Fe-2S]-[ferredoxin] + H2OCOFACTOR Cofactor biosynthesis; ubiquinone biosynthesis.SUBUNIT Component of a multi-subunit COQ enzyme complex, composed of at least COQ3, COQ4, COQ5, COQ6, COQ7 and COQ9. Interacts with COQ8B and COQ7.SUBCELLULAR LOCATION Localizes to cell processes and Golgi apparatus in podocytes.TISSUE SPECIFICITY In the kidney, expressed almost exclusively in glomerular podocytes. In the inner ear, expressed in the spiral ganglion, as well as in stria vascularis and spiral ligament cells.SIMILARITY Belongs to the UbiH/COQ6 family.
created[InstanceEdit:2587579] Weiser, JD
descriptionrecommendedName: fullName evidence="1"Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial ecNumber evidence="1"1.14.15.45 alternativeName: fullName evidence="1"2-methoxy-6-polyprenolphenol 4-hydroxylase ecNumber evidence="1"1.14.15.46 alternativeName: fullName evidence="1"Coenzyme Q10 monooxygenase 6
geneNameCoq6
identifierQ68FU7
isSequenceChangedFALSE
keywordCell projection
FAD
Flavoprotein
Golgi apparatus
Membrane
Mitochondrion
Mitochondrion inner membrane
Monooxygenase
Oxidoreductase
Reference proteome
Transit peptide
Ubiquinone biosynthesis
modified[InstanceEdit:5433710] Weiser, JD
[InstanceEdit:5618415] Weiser, JD
[InstanceEdit:5634237] Weiser, JD
[InstanceEdit:5673015] Weiser, JD
[InstanceEdit:5691543] Weiser, JD
[InstanceEdit:9027688] Weiser, JD
[InstanceEdit:9037114] Weiser, JD
[InstanceEdit:9637257] Weiser, JD
[InstanceEdit:9676415] Weiser, JD
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
[InstanceEdit:9948485] Weiser, Joel, 2025-05-21
[InstanceEdit:9963647] Weiser, Joel, 2025-08-15
nameCoq6
referenceDatabase[ReferenceDatabase:2] UniProt
secondaryIdentifierCOQ6_RAT
sequenceLength476
species[Species:48895] Rattus norvegicus
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