Ubiquinone biosynthesis protein COQ9, mitochondrial , also known as coenzyme Q9 homolog (COQ9), is a protein that in humans is encoded by the COQ9 gene .[ 5]
This locus represents a mitochondrial ubiquinone biosynthesis gene. The encoded protein is likely necessary for biosynthesis of coenzyme Q10 , as mutations at this locus have been associated with autosomal-recessive neonatal-onset primary coenzyme Q10 deficiency.[ 5]
Clinical significance
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This article incorporates text from the United States National Library of Medicine , which is in the public domain .