P97478 (COQ7_MOUSE) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
Ubiquinone biosynthesis protein COQ7 homolog

Alternative name(s):
Coenzyme Q biosynthesis protein 7 homolog
Timing protein clk-1 homolog

Gene names

Name:

Coq7

Organism

Mus musculus (Mouse) [Reference proteome]

Taxonomic identifier

10090 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Euarchontoglires › Glires › Rodentia › Sciurognathi › Muroidea › Muridae › Murinae › Mus › Mus

Protein attributes

Sequence length	217 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence 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.
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 process	Ubiquinone biosynthesis
Cellular component	Membrane Mitochondrion Mitochondrion inner membrane
Domain	Repeat Transit peptide
Ligand	Iron Metal-binding
Technical term	Complete proteome Reference proteome
Gene Ontology (GO)
Biological_process	age-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 ubiquinone biosynthetic process Inferred from mutant phenotype PubMed 11585841PubMed 11716496. Source: MGI
Cellular_component	mitochondrial 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: Compara
Molecular_function	oxidoreductase activity Inferred from electronic annotation. Source: InterPro transition metal ion binding Inferred from electronic annotation. Source: InterPro
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	1 – 34	34	Mitochondrion Potential
	Chain	35 – 217	183	Ubiquinone biosynthesis protein COQ7 homolog		PRO_0000079252
Regions
	Repeat	48 – 129	82	1
	Repeat	130 – 217	88	2
	Region	48 – 217	170	2 X approximate tandem repeats
Sites
	Metal binding	60	1	Iron 1 By similarity
	Metal binding	90	1	Iron 1 By similarity
	Metal binding	90	1	Iron 2 By similarity
	Metal binding	93	1	Iron 1 By similarity
	Metal binding	142	1	Iron 2 By similarity
	Metal binding	178	1	Iron 1 By similarity
	Metal binding	178	1	Iron 2 By similarity
	Metal binding	181	1	Iron 2 By similarity

Sequences

	Sequence		Length	Mass (Da)
	P97478 [UniParc]. Last modified July 15, 1999. Version 3. Checksum: 3BFA05D64BB42ACC	FASTA	217	24,042
	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

References

	« Hide 'large scale' referencesShow '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. , 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.
IPI	IPI00125592.
PIR	JC7756.
RefSeq	NP_034070.1. NM_009940.2.
UniGene	Mm.20634.
3D structure databases
ProteinModelPortal	P97478.
ModBase	Search...
Protein-protein interaction databases
MINT	MINT-1860620.
PTM databases
PhosphoSite	P97478.
Proteomic databases
PaxDb	P97478.
PRIDE	P97478.
Protocols and materials databases
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENSMUST00000032887; ENSMUSP00000032887; ENSMUSG00000030652.
GeneID	12850.
KEGG	mmu:12850.
UCSC	uc009jjy.1. mouse.
Organism-specific databases
CTD	10229.
MGI	MGI:107207. Coq7.
Phylogenomic databases
eggNOG	COG2941.
GeneTree	ENSGT00390000014520.
HOGENOM	HOG000184972.
HOVERGEN	HBG002214.
KO	K06134.
OMA	KAAHACT.
OrthoDB	EOG42V8H8.
Enzyme and pathway databases
UniPathway	UPA00232.
Gene expression databases
ArrayExpress	P97478.
Bgee	P97478.
CleanEx	MM_COQ7.
Genevestigator	P97478.
GermOnline	ENSMUSG00000030652. Mus musculus.
Family and domain databases
Gene3D	1.20.1260.10. 1 hit.
InterPro	IPR009078. Ferritin-like_SF. IPR012347. Ferritin-rel. IPR011566. Ubq_synth_Coq7. [Graphical view]
PANTHER	PTHR11237. PTHR11237. 1 hit.
Pfam	PF03232. COQ7. 1 hit. [Graphical view]
SUPFAM	SSF47240. Ferritin/RR_like. 1 hit.
ProtoNet	Search...
Other
NextBio	282400.
SOURCE	Search...

Entry information

Entry name

COQ7_MOUSE

Accession

Primary (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:	May 29, 2013
This is version 96 of the entry and version 3 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program