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Protein

Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial

Gene

Coq6

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

FAD-dependent monooxygenase required for the C5-ring hydroxylation during ubiquinone biosynthesis. Catalyzes the hydroxylation of 3-polyprenyl-4-hydroxybenzoic acid to 3-polyprenyl-4,5-dihydroxybenzoic acid. The electrons required for the hydroxylation reaction may be funneled indirectly from NADPH via a ferredoxin/ferredoxin reductase system to COQ6.UniRule annotation

Cofactori

FADUniRule annotation

Pathwayi: ubiquinone biosynthesis

This protein is involved in the pathway ubiquinone biosynthesis, which is part of Cofactor biosynthesis.UniRule annotation
View all proteins of this organism that are known to be involved in the pathway ubiquinone biosynthesis and in Cofactor biosynthesis.

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionMonooxygenase, Oxidoreductase
Biological processUbiquinone biosynthesis
LigandFAD, Flavoprotein

Enzyme and pathway databases

UniPathwayiUPA00232

Names & Taxonomyi

Protein namesi
Recommended name:
Ubiquinone biosynthesis monooxygenase COQ6, mitochondrialUniRule annotation (EC:1.14.13.-UniRule annotation)
Alternative name(s):
Coenzyme Q10 monooxygenase 6UniRule annotation
Gene namesi
Name:Coq6UniRule annotation
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 12

Organism-specific databases

MGIiMGI:1924408 Coq6

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Cell projection, Golgi apparatus, Membrane, Mitochondrion, Mitochondrion inner membrane

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 35MitochondrionUniRule annotationAdd BLAST35
ChainiPRO_000020758436 – 476Ubiquinone biosynthesis monooxygenase COQ6, mitochondrialAdd BLAST441

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei219N6-succinyllysineCombined sources1

Proteomic databases

EPDiQ8R1S0
MaxQBiQ8R1S0
PaxDbiQ8R1S0
PeptideAtlasiQ8R1S0
PRIDEiQ8R1S0

PTM databases

iPTMnetiQ8R1S0
PhosphoSitePlusiQ8R1S0

Expressioni

Tissue specificityi

Expressed in the kidney, in podocytes.1 Publication

Developmental stagei

At 12.5 dpc, expressed in the metanephric mesenchyme. At 16.5 dpc, expressed in the condensing metanephric mesenchyme surrounding the ureter tips. At 18.5 dpc, expressed in whole kidney.1 Publication

Gene expression databases

BgeeiENSMUSG00000021235
CleanExiMM_COQ6
ExpressionAtlasiQ8R1S0 baseline and differential
GenevisibleiQ8R1S0 MM

Interactioni

Subunit structurei

Component of a multi-subunit COQ enzyme complex, composed of at least COQ3, COQ4, COQ5, COQ6, COQ7 and COQ9. Interacts with COQ8B and COQ7.UniRule annotation

Protein-protein interaction databases

IntActiQ8R1S0, 1 interactor
MINTiQ8R1S0
STRINGi10090.ENSMUSP00000021661

Structurei

3D structure databases

ProteinModelPortaliQ8R1S0
SMRiQ8R1S0
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the UbiH/COQ6 family.UniRule annotation

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG3855 Eukaryota
COG0654 LUCA
GeneTreeiENSGT00390000015152
HOGENOMiHOG000255772
HOVERGENiHBG029533
InParanoidiQ8R1S0
KOiK06126
OMAiCHPVGGQ
OrthoDBiEOG091G0NNO
PhylomeDBiQ8R1S0
TreeFamiTF105772

Family and domain databases

Gene3Di3.50.50.60, 2 hits
HAMAPiMF_03193 COQ6_monooxygenase, 1 hit
InterProiView protein in InterPro
IPR002938 FAD-bd
IPR036188 FAD/NAD-bd_sf
IPR018168 Ubi_Hdrlase_CS
IPR010971 UbiH/COQ6
IPR000689 UbQ_mOase_COQ6
PfamiView protein in Pfam
PF01494 FAD_binding_3, 2 hits
SUPFAMiSSF51905 SSF51905, 2 hits
TIGRFAMsiTIGR01989 COQ6, 1 hit
TIGR01988 Ubi-OHases, 1 hit
PROSITEiView protein in PROSITE
PS01304 UBIH, 1 hit

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

Q8R1S0-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAARIGSMAG LLCVRWWSSA QLAARGGPLV ASQRWAGSSA DTVYDVVVSG
60 70 80 90 100
GGLVGSAMAC ALGHDIHFHD KKILLLEAGP KKALEKLSET YSNRVSSISP
110 120 130 140 150
GSTTLLSSFG AWDHICNMRC KAFRRMQVWD SCSEALIMFD RDNLDDMGYI
160 170 180 190 200
VENDVIMYAL TKQLEAVADR VKVLYESKAV GYSWPGAFSM ADSSPWVHIT
210 220 230 240 250
LGDGSTLQTK LLIGADGHKS GVRQAAGIQN VSWKYDQSAV VATLHLSEAT
260 270 280 290 300
ENNVAWQRFL PSGPIALLPL SDTLSSLVWS TSHEHAAELV SMDEEEFVDA
310 320 330 340 350
INSAFWSDVH HTDFVDSASA MVRHAVALLK PTKVSARQLP PSIAKVDAKS
360 370 380 390 400
RALFPLGLGH AAEYVRPRVA LIGDAAHRIH PLAGQGVNMG FGDISSLVHH
410 420 430 440 450
LSTAAFNGKD LGSMSHLTGY ETDRQRHNTA LLAATDLLKR LYSTSATPLV
460 470
LLRTWGLQAT NAVSPLKEQI MAFASK
Length:476
Mass (Da):51,393
Last modified:September 5, 2012 - v3
Checksum:iB431032EFE97D9E5
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti33Q → R in AAH51055 (PubMed:15489334).Curated1
Sequence conflicti441L → I in BAC37039 (PubMed:16141072).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK077863 mRNA Translation: BAC37039.1
AK131889 mRNA Translation: BAE20853.1
AK167380 mRNA Translation: BAE39473.1
AC120402 Genomic DNA No translation available.
CH466590 Genomic DNA Translation: EDL02790.1
BC024135 mRNA Translation: AAH24135.1
BC051055 mRNA Translation: AAH51055.1
BC132168 mRNA Translation: AAI32169.1
BC132170 mRNA Translation: AAI32171.1
CCDSiCCDS26044.1
RefSeqiNP_766170.2, NM_172582.3
XP_006515762.1, XM_006515699.2
UniGeneiMm.280062

Genome annotation databases

EnsembliENSMUST00000021661; ENSMUSP00000021661; ENSMUSG00000021235
ENSMUST00000110276; ENSMUSP00000105905; ENSMUSG00000021235
GeneIDi217707
KEGGimmu:217707
UCSCiuc007ofb.2 mouse

Similar proteinsi

Entry informationi

Entry nameiCOQ6_MOUSE
AccessioniPrimary (citable) accession number: Q8R1S0
Secondary accession number(s): Q3TJM2, Q80V13, Q8BJY5
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 28, 2002
Last sequence update: September 5, 2012
Last modified: May 23, 2018
This is version 129 of the entry and version 3 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

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