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Coenzyme Q and Phenylketonuria

Abstract

INVESTIGATIONS by Olson's group1,2 of the biosynthesis of coenzyme Q9 (ubiquinone-45) in the rat have shown that phenylalanine is a metabolic precursor of the benzoquinoid ring of coenzyme Q. Presumably, hydroxylation of phenylalanine to yield tyrosine would be an intermediate step in the biosynthesis of coenzyme Q (ref. 3). If tyrosine is such an obligatory intermediate, the question is raised whether a deficiency of coenzyme Q may occur in phenylketonuria, since impairment of the hydroxylation of phenylalanine to tyrosine in this disease has been demonstrated4.

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References

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NAPIER, E., KREYDEN, R., HENLEY, K. et al. Coenzyme Q and Phenylketonuria. Nature 202, 806–807 (1964). https://doi.org/10.1038/202806a0

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