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HAMAP rule MF_03193

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General rule information [?]

PURL https://purl.expasy.org/hamap/rule/MF_03193
Accession MF_03193
Dates 20-MAR-2015 (Created)
01-APR-2025 (Last updated, Version 13)
Name COQ6_monooxygenase
Scope(s) Eukaryota
Template(s) P53318; Q9Y2Z9; [ Recover all ]
Triggered by HAMAP; MF_03193 (Get profile general information and statistics)

Propagated annotation [?]

Identifier, protein and gene names [?]

Identifier COQ6
Protein name RecName: Full=Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial;
                 EC=1.14.15.45;
AltName: Full=2-methoxy-6-polyprenolphenol 4-hydroxylase;
                 EC=1.14.15.46;
case <OC:Vertebrata>
Protein name + AltName: Full=Coenzyme Q10 monooxygenase 6;
end case
case <OC:Saccharomyces>
Protein name + AltName: Full=Ubiquinone C4-deaminase;
                 EC=3.5.-.-;
end case
case <OC:Vertebrata> or <OC:Fungi> or <OC:Viridiplantae>
Gene name Name=COQ6;
else case <OC:Drosophilidae>
Gene name Name=Coq6;
else case <OC:Insecta> or <OC:Dictyostelia>
Gene name Name=coq6;
else case <OC:Caenorhabditis>
Gene name Name=coq-6;
end case

Comments [?]

case <OC:Mammalia>
FUNCTIONFAD-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 Reaction=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] + H2O; Xref=Rhea:RHEA:81259, Rhea:RHEA-COMP:10000, Rhea:RHEA-COMP:10001, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:15379, ChEBI:CHEBI:33737, ChEBI:CHEBI:33738, ChEBI:CHEBI:62793, ChEBI:CHEBI:84503; EC=1.14.15.45;
CATALYTIC ACTIVITY Reaction=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] + H2O; Xref=Rhea:RHEA:81295, Rhea:RHEA-COMP:10000, Rhea:RHEA-COMP:10001, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:15379, ChEBI:CHEBI:33737, ChEBI:CHEBI:33738, ChEBI:CHEBI:50774, ChEBI:CHEBI:64180; EC=1.14.15.46;
else case <OC:Saccharomyces>
FUNCTIONFAD-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-hexaprenyl)benzoic acid to 3,4- dihydroxy-5-(all-trans-hexaprenyl)benzoic acid. Also acts downstream of COQ4, for the C1-hydroxylation during ubiquinone biosynthesis by catalyzing the hydroxylation of 2-methoxy-6-(all-trans- hexaprenyl)phenol to 2-methoxy-6-(all-trans-hexaprenyl)benzene-1,4- diol. The electrons required for the hydroxylation reaction are funneled indirectly from NADPH via ferredoxin (YAH1) and ferredoxin reductase (ARH1) to COQ6. Can also convert 3-hexaprenyl-4-aminobenzoic acid (HAB), a COQ2-prenylated pABA, to DHHB in a two step process. HAB is first hydroxylated at C5 to yield 3-hexaprenyl-4-amino-5- hydroxybenzoic acid (HHAB) which is further deaminated at C4 by COQ6 to produce DHHB.
CATALYTIC ACTIVITY Reaction=4-hydroxy-3-(all-trans-hexaprenyl)benzoate + 2 reduced [2Fe- 2S]-[ferredoxin] + O2 + 2 H(+) = 3,4-dihydroxy-5-(all-trans- hexaprenyl)benzoate + 2 oxidized [2Fe-2S]-[ferredoxin] + H2O; Xref=Rhea:RHEA:20361, Rhea:RHEA-COMP:10000, Rhea:RHEA-COMP:10001, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:15379, ChEBI:CHEBI:33737, ChEBI:CHEBI:33738, ChEBI:CHEBI:58373, ChEBI:CHEBI:84492; EC=1.14.15.45;
CATALYTIC ACTIVITY Reaction=2-methoxy-6-(all-trans-hexaprenyl)phenol + 2 reduced [2Fe-2S]- [ferredoxin] + O2 + 2 H(+) = 2-methoxy-6-(all-trans- hexaprenyl)benzene-1,4-diol + 2 oxidized [2Fe-2S]-[ferredoxin] + H2O; Xref=Rhea:RHEA:81279, Rhea:RHEA-COMP:10000, Rhea:RHEA-COMP:10001, ChEBI:CHEBI:1109, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:15379, ChEBI:CHEBI:33737, ChEBI:CHEBI:33738, ChEBI:CHEBI:61472; EC=1.14.15.46;
CATALYTIC ACTIVITY Reaction=4-amino-3-(all-trans-hexaprenyl)benzoate + 2 reduced [2Fe-2S]- [ferredoxin] + O2 + 2 H(+) = 4-amino-5-hydroxy-3-(all-trans- hexaprenyl)benzoate + 2 oxidized [2Fe-2S]-[ferredoxin] + H2O; Xref=Rhea:RHEA:58392, Rhea:RHEA-COMP:10000, Rhea:RHEA-COMP:10001, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:15379, ChEBI:CHEBI:33737, ChEBI:CHEBI:33738, ChEBI:CHEBI:142618, ChEBI:CHEBI:142619;
CATALYTIC ACTIVITY Reaction=4-amino-5-hydroxy-3-(all-trans-hexaprenyl)benzoate + 4 reduced [2Fe-2S]-[ferredoxin] + O2 + 5 H(+) = 3,4-dihydroxy-5-(all-trans- hexaprenyl)benzoate + 4 oxidized [2Fe-2S]-[ferredoxin] + NH4(+) + H2O; Xref=Rhea:RHEA:58396, Rhea:RHEA-COMP:10000, Rhea:RHEA- COMP:10001, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:15379, ChEBI:CHEBI:28938, ChEBI:CHEBI:33737, ChEBI:CHEBI:33738, ChEBI:CHEBI:58373, ChEBI:CHEBI:142619;
else case <OC:Schizosaccharomyces>
FUNCTIONFAD-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.
CATALYTIC ACTIVITY Reaction=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] + H2O; Xref=Rhea:RHEA:81259, Rhea:RHEA-COMP:10000, Rhea:RHEA-COMP:10001, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:15379, ChEBI:CHEBI:33737, ChEBI:CHEBI:33738, ChEBI:CHEBI:62793, ChEBI:CHEBI:84503; EC=1.14.15.45;
CATALYTIC ACTIVITY Reaction=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] + H2O; Xref=Rhea:RHEA:81295, Rhea:RHEA-COMP:10000, Rhea:RHEA-COMP:10001, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:15379, ChEBI:CHEBI:33737, ChEBI:CHEBI:33738, ChEBI:CHEBI:50774, ChEBI:CHEBI:64180; EC=1.14.15.46;
else
FUNCTIONFAD-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-polyprenyl)benzoic acid to 3,4- dihydroxy-5-(all-trans-polyprenyl)benzoic acid. Also acts downstream of coq4, for the C1-hydroxylation during ubiquinone biosynthesis by catalyzing the hydroxylation of 2-methoxy-6-(all-trans- polyprenyl)phenol to 2-methoxy-6-(all-trans-polyprenyl)benzene-1,4- diol. The electrons required for the hydroxylation reaction are funneled indirectly to coq6 from NADPH via a ferredoxin/ferredoxin reductase system.
CATALYTIC ACTIVITY Reaction=a 4-hydroxy-3-(all-trans-polyprenyl)benzoate + 2 reduced [2Fe- 2S]-[ferredoxin] + O2 + 2 H(+) = a 3,4-dihydroxy-5-(all-trans- polyprenyl)benzoate + 2 oxidized [2Fe-2S]-[ferredoxin] + H2O; Xref=Rhea:RHEA:81195, Rhea:RHEA-COMP:9514, Rhea:RHEA-COMP:10000, Rhea:RHEA-COMP:10001, Rhea:RHEA-COMP:10930, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:15379, ChEBI:CHEBI:33737, ChEBI:CHEBI:33738, ChEBI:CHEBI:64694, ChEBI:CHEBI:78396; EC=1.14.15.45;
CATALYTIC ACTIVITY Reaction=a 2-methoxy-6-(all-trans-polyprenyl)phenol + 2 reduced [2Fe- 2S]-[ferredoxin] + O2 + 2 H(+) = a 2-methoxy-6-(all-trans- polyprenyl)benzene-1,4-diol + 2 oxidized [2Fe-2S]-[ferredoxin] + H2O; Xref=Rhea:RHEA:81183, Rhea:RHEA-COMP:9551, Rhea:RHEA-COMP:10000, Rhea:RHEA-COMP:10001, Rhea:RHEA-COMP:10858, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:15379, ChEBI:CHEBI:33737, ChEBI:CHEBI:33738, ChEBI:CHEBI:62731, ChEBI:CHEBI:84166; EC=1.14.15.46;
end case
COFACTOR Name=FAD; Xref=ChEBI:CHEBI:57692;
PATHWAYCofactor biosynthesis; ubiquinone biosynthesis.
case <OC:Vertebrata>
SUBUNITComponent of a multi-subunit COQ enzyme complex, composed of at least @gn(COQ3), @gn(COQ4), @gn(COQ5), @gn(COQ6), @gn(COQ7) and @gn(COQ9). Interacts with @gn(COQ8B) and @gn(COQ7).
else case <OC:Fungi>
SUBUNITComponent of a multi-subunit COQ enzyme complex, composed of at least @gn(COQ3), @gn(COQ4), @gn(COQ5), @gn(COQ6), @gn(COQ7) and @gn(COQ9).
else
SUBUNITComponent of a multi-subunit COQ enzyme complex.
end case
case <OC:Vertebrata>
SUBCELLULAR LOCATIONMitochondrion inner membrane; Peripheral membrane protein; Matrix side. Golgi apparatus. Cell projection. Note=Localizes to cell processes and Golgi apparatus in podocytes.
else
SUBCELLULAR LOCATIONMitochondrion inner membrane; Peripheral membrane protein; Matrix side.
end case
case not <AnyFeature:TransitM>
MISCELLANEOUSThis protein may be expected to contain an N-terminal transit peptide but none has been predicted.
end case
SIMILARITYBelongs to the UbiH/COQ6 family.

Keywords [?]

FAD
Flavoprotein
Membrane
Mitochondrion
Mitochondrion inner membrane
Monooxygenase
Oxidoreductase
Ubiquinone biosynthesis
case <OC:Vertebrata>
Cell projection
Golgi apparatus
end case

Gene Ontology [?]

GO:0031314; Cellular component:extrinsic component of mitochondrial inner membrane
case <OCellular component:Vertebrata>
GO:0042995; Cellular component:cell projection
GO:0005794; Cellular component:Golgi apparatus
end case
GO:0016712; Molecular function: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
GO:0006744; Biological process:ubiquinone biosynthetic process

Cross-references [?]

PROSITE PS01304; UBIH; 1;
Pfam PF01494; FAD_binding_3; 1;
PRINTS PR00420; RNGMNOXGNASE; 1;
NCBIfam TIGR01989; COQ6; 1;
NCBIfam TIGR01988; Ubi-OHases; 1;
General TransitM; -; 0-1;

Features [?]


Additional information [?]

Size range 451-605 amino acids
Related rules None
Fusion Nter: None Cter: None



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