Target type: molecularfunction
Catalysis of the reaction: 7,8-dihydrobiopterin + NADP+ = sepiapterin + NADPH + H+. [EC:1.1.1.153]
Sepiapterin reductase (SPR) catalyzes the NADPH-dependent reduction of 7,8-dihydrobiopterin to tetrahydrobiopterin (BH4). BH4 is a cofactor for several enzymes including phenylalanine hydroxylase, tyrosine hydroxylase, and tryptophan hydroxylase. These enzymes are involved in the biosynthesis of neurotransmitters such as dopamine, norepinephrine, and serotonin. SPR is therefore essential for the proper functioning of the nervous system. SPR also plays a role in the metabolism of folate and purines. Mutations in the SPR gene can cause a variety of disorders including hyperphenylalaninemia, severe combined immunodeficiency (SCID), and autism spectrum disorder.'
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| Protein | Definition | Taxonomy |
|---|---|---|
| Sepiapterin reductase | A sepiapterin reductase that is encoded in the genome of human. [PRO:DNx, UniProtKB:P35270] | Homo sapiens (human) |
| Compound | Definition | Classes | Roles |
|---|---|---|---|
| n-acetylserotonin | N-acetylserotonin : An N-acylserotonin resulting from the formal condensation of the primary amino group of serotonin with the carboxy group of acetic acid. | acetamides; N-acylserotonin; phenols | antioxidant; human metabolite; mouse metabolite; tropomyosin-related kinase B receptor agonist |