Abstract
The phenylalanine hydroxylating system is complex, consisting of three enzymes and two coenzymes1,2,3. One of the coenzymes, NADH, plays a near-ubiquitous role in intermediary metabolism. By contrast, the other cofactor, tetrahydrobiopterin (BH4), discovered during our early studies on the characterization of the phenylalanine hydroxylating system,1 plays a unique role as the essential coenzyme for certain hydroxylases such as the enzymes that catalyze the hydroxylation of the aromatic amino acids, phenylalanine, tyrosine and tryptophan3.
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Kaufman, S., Citron, B.A., Davis, M., Milstien, S. (1993). The Isolation and Characterization of Clones of 4a-Hydroxytetrahydrobiopterin Dehydratase. In: Ayling, J.E., Nair, M.G., Baugh, C.M. (eds) Chemistry and Biology of Pteridines and Folates. Advances in Experimental Medicine and Biology, vol 338. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-2960-6_19
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DOI: https://doi.org/10.1007/978-1-4615-2960-6_19
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