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Tetrahydrobiopterin (BH4) biosynthesis is a critical metabolic pathway responsible for the production of BH4, an essential enzymatic cofactor for several enzymes, particularly those involved in amino acid metabolism, neurotransmitter synthesis, and nitric oxide production. The pathway includes de novo synthesis from GTP, a salvage pathway, and a recycling pathway. Deficiencies in the pathway can lead to various diseases, including hyperphenylalaninemia and neurological disorders.
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