The protein encoded by this gene is similar to a mitochondrial di-iron containing hydroxylase in Saccharomyces cerevisiae that is involved with ubiquinone biosynthesis. Mutations in the yeast gene lead to slower development and longer life span. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jul 2010]
Function:
Involved in lifespan determination in ubiquinone-independent manner. Involved in ubiquinone biosynthesis. Potential central metabolic regulator.
Subcellular Location:
Mitochondrion inner membrane. 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.
Tissue Specificity:
Expressed dominantly in heart and skeletal muscle.
Similarity:
Belongs to the COQ7 family.
SWISS:
Q91967
Gene ID:
10229
Database links:
Entrez Gene: 10229 Human
Omim: 601683 Human
SwissProt: Q91967 Human
SwissProt: Q9BTT7 Human
Unigene: 157113 Human
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