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P97478 (COQ7_MOUSE) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 105. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (3) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Ubiquinone biosynthesis protein COQ7 homolog
Alternative name(s):
Coenzyme Q biosynthesis protein 7 homolog
Timing protein clk-1 homolog
Gene names
Name:Coq7
OrganismMus musculus (Mouse) [Reference proteome]
Taxonomic identifier10090 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus

Protein attributes

Sequence length217 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at transcript level

General annotation (Comments)

Function

Involved in lifespan determination in ubiquinone-independent manner. Involved in ubiquinone biosynthesis. Potential central metabolic regulator. Ref.9

Cofactor

Binds 2 iron ions per subunit By similarity.

Pathway

Cofactor biosynthesis; ubiquinone biosynthesis.

Subunit structure

Interacts with ADCK4 and COQ6 By similarity.

Subcellular location

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side. Note: Synthesized as a preprotein that is imported into the mitochondrial matrix, where the sequence is cleaved off and the mature protein becomes loosely associated with the inner membrane. Ref.8

Tissue specificity

Highly expressed in tissues with high energy demand such as heart, muscle, liver, and kidney. Ref.7

Disruption phenotype

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. Ref.9

Miscellaneous

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

Sequence similarities

Belongs to the COQ7 family.

Ontologies

Keywords
   Biological processUbiquinone biosynthesis
   Cellular componentMembrane
Mitochondrion
Mitochondrion inner membrane
   DomainRepeat
Transit peptide
   LigandIron
Metal-binding
   Technical termComplete proteome
Reference proteome
Gene Ontology (GO)
   Biological_processage-dependent response to oxidative stress

Inferred from mutant phenotype Ref.9. Source: MGI

cellular response to oxidative stress

Inferred from mutant phenotype PubMed 18635541. Source: MGI

determination of adult lifespan

Inferred from mutant phenotype Ref.9. Source: MGI

in utero embryonic development

Inferred from mutant phenotype PubMed 11585841PubMed 11716496. Source: MGI

mitochondrial ATP synthesis coupled electron transport

Inferred from mutant phenotype PubMed 18635541. Source: MGI

mitochondrion morphogenesis

Inferred from mutant phenotype PubMed 11716496. Source: MGI

neural tube formation

Inferred from mutant phenotype PubMed 11716496. Source: MGI

neurogenesis

Inferred from mutant phenotype PubMed 11716496. Source: MGI

respiratory electron transport chain

Inferred from mutant phenotype PubMed 11585841Ref.9. Source: MGI

response to oxidative stress

Inferred from mutant phenotype Ref.9. Source: MGI

ubiquinone biosynthetic process

Inferred from mutant phenotype PubMed 11585841PubMed 11716496. Source: MGI

   Cellular_componentmitochondrial inner membrane

Inferred from electronic annotation. Source: UniProtKB-SubCell

mitochondrion

Inferred from direct assay PubMed 14651853PubMed 18614015. Source: MGI

nucleus

Inferred from electronic annotation. Source: Ensembl

   Molecular_functionmetal ion binding

Inferred from electronic annotation. Source: UniProtKB-KW

Complete GO annotation...

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Transit peptide1 – 3434Mitochondrion Potential
Chain35 – 217183Ubiquinone biosynthesis protein COQ7 homolog
PRO_0000079252

Regions

Repeat48 – 129821
Repeat130 – 217882
Region48 – 2171702 X approximate tandem repeats

Sites

Metal binding601Iron 1 By similarity
Metal binding901Iron 1 By similarity
Metal binding901Iron 2 By similarity
Metal binding931Iron 1 By similarity
Metal binding1421Iron 2 By similarity
Metal binding1781Iron 1 By similarity
Metal binding1781Iron 2 By similarity
Metal binding1811Iron 2 By similarity

Sequences

Sequence LengthMass (Da)Tools
P97478 [UniParc].

Last modified July 15, 1999. Version 3.
Checksum: 3BFA05D64BB42ACC

FASTA21724,042
        10         20         30         40         50         60 
MSAAGAIAAA SVGRLRTGVR RPFSEYGRGL IIRCHSSGMT LDNINRAAVD RIIRVDHAGE 

        70         80         90        100        110        120 
YGANRIYAGQ MAVLGRTSVG PVIQKMWDQE KNHLKKFNEL MIAFRVRPTV LMPLWNVAGF 

       130        140        150        160        170        180 
ALGAGTALLG KEGAMACTVA VEESIANHYN NQIRMLMEED PEKYEELLQV IKQFRDEELE 

       190        200        210 
HHDTGLDHDA ELAPAYALLK RIIQAGCSAA IYLSERF 

« Hide

References

« Hide 'large scale' references
[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]Marra M., Hillier L., Allen M., Bowles M., Dietrich N., Dubuque T., Geisel S., Kucaba T., Lacy M., Le M., Martin J., Morris M., Schellenberg K., Steptoe M., Tan F., Underwood K., Moore B., Theising B. expand/collapse author list , Wylie T., Lennon G., Soares B., Wilson R., Waterston R.
Submitted (AUG-1996) to the EMBL/GenBank/DDBJ databases
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.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
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.
CCDSCCDS21771.1.
PIRJC7756.
RefSeqNP_034070.1. NM_009940.2.
UniGeneMm.20634.

3D structure databases

ProteinModelPortalP97478.
SMRP97478. Positions 49-109.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

MINTMINT-1860620.

PTM databases

PhosphoSiteP97478.

Proteomic databases

MaxQBP97478.
PaxDbP97478.
PRIDEP97478.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENSMUST00000032887; ENSMUSP00000032887; ENSMUSG00000030652.
GeneID12850.
KEGGmmu:12850.
UCSCuc009jjy.1. mouse.

Organism-specific databases

CTD10229.
MGIMGI:107207. Coq7.

Phylogenomic databases

eggNOGCOG2941.
GeneTreeENSGT00390000014520.
HOGENOMHOG000184972.
HOVERGENHBG002214.
KOK06134.
OMAREMEGHE.
PhylomeDBP97478.
TreeFamTF314559.

Enzyme and pathway databases

UniPathwayUPA00232.

Gene expression databases

ArrayExpressP97478.
BgeeP97478.
CleanExMM_COQ7.
GenevestigatorP97478.

Family and domain databases

Gene3D1.20.1260.10. 1 hit.
InterProIPR009078. Ferritin-like_SF.
IPR012347. Ferritin-rel.
IPR011566. Ubq_synth_Coq7.
[Graphical view]
PANTHERPTHR11237. PTHR11237. 1 hit.
PfamPF03232. COQ7. 1 hit.
[Graphical view]
SUPFAMSSF47240. SSF47240. 1 hit.
ProtoNetSearch...

Other

NextBio282400.
PROP97478.
SOURCESearch...

Entry information

Entry nameCOQ7_MOUSE
AccessionPrimary (citable) accession number: P97478
Secondary accession number(s): Q9R0D7
Entry history
Integrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: July 15, 1999
Last modified: July 9, 2014
This is version 105 of the entry and version 3 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Relevant documents

SIMILARITY comments

Index of protein domains and families

PATHWAY comments

Index of metabolic and biosynthesis pathways

MGD cross-references

Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot