LMPD Database

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LMP000820

UniProt Annotations

Entry Information
Gene Namedemethyl-Q 7
Protein EntryCOQ7_MOUSE
UniProt IDP97478
SpeciesMouse
Comments
Comment typeDescription
CofactorName=Fe cation; Xref=ChEBI:CHEBI:24875; Evidence={ECO:0000250}; Note=Binds 2 iron ions per subunit. {ECO:0000250};
Disruption PhenotypeMice 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. {ECO:0000269|PubMed:19478076}.
FunctionInvolved in lifespan determination in ubiquinone- independent manner. Involved in ubiquinone biosynthesis. Potential central metabolic regulator. {ECO:0000269|PubMed:19478076}.
MiscellaneousIn life-span analysis, transgenic expression reverted the extended life span of clk-1 to the comparable level with wild-type control.
PathwayCofactor biosynthesis; ubiquinone biosynthesis.
SimilarityBelongs to the COQ7 family. {ECO:0000305}.
Subcellular LocationMitochondrion inner membrane {ECO:0000269|PubMed:11387338}; Peripheral membrane protein {ECO:0000269|PubMed:11387338}; Matrix side {ECO:0000269|PubMed:11387338}. 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.
SubunitInteracts with ADCK4 and COQ6. {ECO:0000250}.
Tissue SpecificityHighly expressed in tissues with high energy demand such as heart, muscle, liver, and kidney. {ECO:0000269|PubMed:11511092}.