Biology:Dihydrobiopterin reductase
Dihydrobiopterin reductase is an enzyme that catalyzes the reversible formation of tetrahydrobiopterin from dihydrobiopterin using NADPH. A deficiency of this enzyme can result in malignant hyperphenylalaninemia.[1] Dihydrobiopterin is a compound produced in the synthesis of dopamine, norepinephrine and epinephrine through production of the intermediate L-3,4-dihydroxyphenylalanine, also known as L-DOPA. Phenylalanine is converted into dopa using tetrahydrobiopterin releasing dihydrobiopterin and water, which is then converted back into tetrahydrobiopterin using the NADPH-dependent enzyme dihydrobiopterin reductase.
Deficiency
A deficiency of dihydrobiopterin reductase is one cause of tetrahydrobiopterin deficiency. Dihydrobiopterin reductase deficiency is treated with tyrosine supplements, a controlled diet which is lacking in phenylalanine,[2] well as supplementation of L-DOPA.
See also
References
- ↑ "Dihydropteridine reductase". http://medical-dictionary.thefreedictionary.com/dihydrobiopterin+reductase.
- ↑ Pawlina, Wojciech; Ross, Michael W. (2006). Histology: a text and atlas: with correlated cell and molecular biology. Philadelphia: Lippincott Wiliams & Wilkins. ISBN 0-7817-5056-3.
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