Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

5-demethoxyubiquinone hydroxylase, mitochondrial

Gene

Coq7

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at transcript leveli

Functioni

Catalyzes the hydroxylation of 2-polyprenyl-3-methyl-6-methoxy-1,4-benzoquinol (DMQH2) during ubiquinone biosynthesis. Has also a structural role in the COQ enzyme complex, stabilizing other COQ polypeptides (By similarity). Involved in lifespan determination in a ubiquinone-independent manner (PubMed:19478076).UniRule annotation1 Publication

Cofactori

Fe cationUniRule annotationNote: Binds 2 iron ions per subunit.UniRule annotation

Pathway: 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.

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Metal bindingi60 – 601Iron 1UniRule annotation
Metal bindingi90 – 901Iron 1UniRule annotation
Metal bindingi90 – 901Iron 2UniRule annotation
Metal bindingi93 – 931Iron 1UniRule annotation
Metal bindingi142 – 1421Iron 2UniRule annotation
Metal bindingi178 – 1781Iron 1UniRule annotation
Metal bindingi178 – 1781Iron 2UniRule annotation
Metal bindingi181 – 1811Iron 2UniRule annotation

GO - Molecular functioni

GO - Biological processi

  • age-dependent response to oxidative stress Source: MGI
  • cellular response to oxidative stress Source: MGI
  • determination of adult lifespan Source: MGI
  • in utero embryonic development Source: MGI
  • mitochondrial ATP synthesis coupled electron transport Source: MGI
  • mitochondrion morphogenesis Source: MGI
  • neural tube formation Source: MGI
  • neurogenesis Source: MGI
  • respiratory electron transport chain Source: MGI
  • response to oxidative stress Source: MGI
  • ubiquinone biosynthetic process Source: MGI
Complete GO annotation...

Keywords - Molecular functioni

Monooxygenase, Oxidoreductase

Keywords - Biological processi

Ubiquinone biosynthesis

Keywords - Ligandi

Iron, Metal-binding

Enzyme and pathway databases

ReactomeiREACT_309628. Ubiquinol biosynthesis.
UniPathwayiUPA00232.

Names & Taxonomyi

Protein namesi
Recommended name:
5-demethoxyubiquinone hydroxylase, mitochondrialUniRule annotation (EC:1.14.13.-UniRule annotation)
Short name:
DMQ hydroxylaseUniRule annotation
Alternative name(s):
Timing protein clk-1 homologUniRule annotation
Ubiquinone biosynthesis monooxygenase COQ7UniRule annotation
Gene namesi
Name:Coq7UniRule annotation
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
ProteomesiUP000000589 Componenti: Chromosome 7

Organism-specific databases

MGIiMGI:107207. Coq7.

Subcellular locationi

GO - Cellular componenti

  • mitochondrial inner membrane Source: GO_Central
  • mitochondrion Source: MGI
  • nucleus Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Disruption phenotypei

Mice lacking Coq7 start to die after E8. Heterozygous mutant reveal that the reduction of Coq7 levels in these animals profoundly alters their mitochondrial function despite the fact that ubiquinone production is unaffected. The mitochondria of young mutants heterozygous are dysfunctional, exhibiting reduced energy metabolism and a substantial increase in oxidative stress.1 Publication

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 2323MitochondrionSequence AnalysisAdd
BLAST
Chaini24 – 2171945-demethoxyubiquinone hydroxylase, mitochondrialPRO_0000079252Add
BLAST

Proteomic databases

MaxQBiP97478.
PaxDbiP97478.
PRIDEiP97478.

PTM databases

PhosphoSiteiP97478.

Expressioni

Tissue specificityi

Highly expressed in tissues with high energy demand such as heart, muscle, liver, and kidney.1 Publication

Gene expression databases

BgeeiP97478.
CleanExiMM_COQ7.
ExpressionAtlasiP97478. baseline and differential.
GenevisibleiP97478. 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 ADCK4 and COQ6. Interacts with COQ9.UniRule annotation

Protein-protein interaction databases

MINTiMINT-1860620.
STRINGi10090.ENSMUSP00000032887.

Structurei

3D structure databases

ProteinModelPortaliP97478.
SMRiP97478. Positions 49-109.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Repeati48 – 129821Add
BLAST
Repeati130 – 217882Add
BLAST

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni48 – 2171702 X approximate tandem repeatsAdd
BLAST

Sequence similaritiesi

Belongs to the COQ7 family.UniRule annotation

Keywords - Domaini

Repeat, Transit peptide

Phylogenomic databases

eggNOGiCOG2941.
GeneTreeiENSGT00390000014520.
HOGENOMiHOG000184972.
HOVERGENiHBG002214.
InParanoidiP97478.
KOiK06134.
OMAiEGHEQVH.
PhylomeDBiP97478.
TreeFamiTF314559.

Family and domain databases

Gene3Di1.20.1260.10. 1 hit.
HAMAPiMF_01658. COQ7.
InterProiIPR009078. Ferritin-like_SF.
IPR012347. Ferritin-rel.
IPR011566. Ubq_synth_Coq7.
[Graphical view]
PANTHERiPTHR11237. PTHR11237. 1 hit.
PfamiPF03232. COQ7. 1 hit.
[Graphical view]
SUPFAMiSSF47240. SSF47240. 1 hit.

Sequencei

Sequence statusi: Complete.

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

P97478-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MSAAGAIAAA SVGRLRTGVR RPFSEYGRGL IIRCHSSGMT LDNINRAAVD
60 70 80 90 100
RIIRVDHAGE YGANRIYAGQ MAVLGRTSVG PVIQKMWDQE KNHLKKFNEL
110 120 130 140 150
MIAFRVRPTV LMPLWNVAGF ALGAGTALLG KEGAMACTVA VEESIANHYN
160 170 180 190 200
NQIRMLMEED PEKYEELLQV IKQFRDEELE HHDTGLDHDA ELAPAYALLK
210
RIIQAGCSAA IYLSERF
Length:217
Mass (Da):24,042
Last modified:July 15, 1999 - v3
Checksum:i3BFA05D64BB42ACC
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF080580 mRNA. Translation: AAC31572.1.
AF098949 mRNA. Translation: AAD43649.1.
BC038681 mRNA. Translation: AAH38681.1.
AF053770 mRNA. Translation: AAC69179.1.
U81277 mRNA. Translation: AAC53055.1.
AA030846 mRNA. No translation available.
CCDSiCCDS21771.1.
PIRiJC7756.
RefSeqiNP_034070.1. NM_009940.3.
UniGeneiMm.20634.

Genome annotation databases

EnsembliENSMUST00000032887; ENSMUSP00000032887; ENSMUSG00000030652.
GeneIDi12850.
KEGGimmu:12850.
UCSCiuc009jjy.1. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF080580 mRNA. Translation: AAC31572.1.
AF098949 mRNA. Translation: AAD43649.1.
BC038681 mRNA. Translation: AAH38681.1.
AF053770 mRNA. Translation: AAC69179.1.
U81277 mRNA. Translation: AAC53055.1.
AA030846 mRNA. No translation available.
CCDSiCCDS21771.1.
PIRiJC7756.
RefSeqiNP_034070.1. NM_009940.3.
UniGeneiMm.20634.

3D structure databases

ProteinModelPortaliP97478.
SMRiP97478. Positions 49-109.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

MINTiMINT-1860620.
STRINGi10090.ENSMUSP00000032887.

PTM databases

PhosphoSiteiP97478.

Proteomic databases

MaxQBiP97478.
PaxDbiP97478.
PRIDEiP97478.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000032887; ENSMUSP00000032887; ENSMUSG00000030652.
GeneIDi12850.
KEGGimmu:12850.
UCSCiuc009jjy.1. mouse.

Organism-specific databases

CTDi10229.
MGIiMGI:107207. Coq7.

Phylogenomic databases

eggNOGiCOG2941.
GeneTreeiENSGT00390000014520.
HOGENOMiHOG000184972.
HOVERGENiHBG002214.
InParanoidiP97478.
KOiK06134.
OMAiEGHEQVH.
PhylomeDBiP97478.
TreeFamiTF314559.

Enzyme and pathway databases

UniPathwayiUPA00232.
ReactomeiREACT_309628. Ubiquinol biosynthesis.

Miscellaneous databases

NextBioi282400.
PROiP97478.
SOURCEiSearch...

Gene expression databases

BgeeiP97478.
CleanExiMM_COQ7.
ExpressionAtlasiP97478. baseline and differential.
GenevisibleiP97478. MM.

Family and domain databases

Gene3Di1.20.1260.10. 1 hit.
HAMAPiMF_01658. COQ7.
InterProiIPR009078. Ferritin-like_SF.
IPR012347. Ferritin-rel.
IPR011566. Ubq_synth_Coq7.
[Graphical view]
PANTHERiPTHR11237. PTHR11237. 1 hit.
PfamiPF03232. COQ7. 1 hit.
[Graphical view]
SUPFAMiSSF47240. SSF47240. 1 hit.
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "Cloning and characterization of murine clk-1."
    Kim H.J., Moon Y.I., Lee J.E., Lee H.W., Seo J.S.
    Submitted (JUL-1998) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
    Strain: 129.
  2. "Orthologues of the Caenorhabditis elegans longevity gene clk-1 in mouse and human."
    Asaumi S., Kuroyanagi H., Seki N., Shirasawa T.
    Genomics 58:293-301(1999) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
  3. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Strain: C57BL/6J.
    Tissue: Mammary gland.
  4. "Conservation of the Caenorhabditis elegans timing gene clk-1 from yeast to human: a gene required for ubiquinone biosynthesis with potential implications for aging."
    Vajo Z., King L.M., Jonassen T., Wilkin D.J., Ho N., Munnich A., Clarke C.F., Francomano C.A.
    Mamm. Genome 10:1000-1004(1999) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 39-217.
  5. "Structural and functional conservation of the Caenorhabditis elegans timing gene clk-1."
    Ewbank J.J., Barnes T.M., Lakowski B., Lussier M., Bussey H., Hekimi S.
    Science 275:980-983(1997) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 74-175.
    Tissue: Embryo.
  6. Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 146-217.
    Strain: C57BL/6J.
    Tissue: Embryo.
  7. "Mouse coq7/clk-1 orthologue rescued slowed rhythmic behavior and extended life span of clk-1 longevity mutant in Caenorhabditis elegans."
    Takahashi M., Asaumi S., Honda S., Suzuki Y., Nakai D., Kuroyanagi H., Shimizu T., Honda Y., Shirasawa T.
    Biochem. Biophys. Res. Commun. 286:534-540(2001) [PubMed] [Europe PMC] [Abstract]
    Cited for: TISSUE SPECIFICITY, TRANSGENIC MICE.
  8. "Mouse CLK-1 is imported into mitochondria by an unusual process that requires a leader sequence but no membrane potential."
    Jiang N., Levavasseur F., McCright B., Shoubridge E.A., Hekimi S.
    J. Biol. Chem. 276:29218-29225(2001) [PubMed] [Europe PMC] [Abstract]
    Cited for: SUBCELLULAR LOCATION.
  9. "Reversal of the mitochondrial phenotype and slow development of oxidative biomarkers of aging in long-lived Mclk1+/- mice."
    Lapointe J., Stepanyan Z., Bigras E., Hekimi S.
    J. Biol. Chem. 284:20364-20374(2009) [PubMed] [Europe PMC] [Abstract]
    Cited for: FUNCTION, DISRUPTION PHENOTYPE.

Entry informationi

Entry nameiCOQ7_MOUSE
AccessioniPrimary (citable) accession number: P97478
Secondary accession number(s): Q9R0D7
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: July 15, 1999
Last modified: June 24, 2015
This is version 115 of the entry and version 3 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Miscellaneous

In life-span analysis, transgenic expression reverted the extended life span of clk-1 to the comparable level with wild-type control.

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.