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Diseases and Pathophysiology Arising from Biopterin Dysregulation
並列摘要
Tetrahydrobiopterin (BH4) is required as cofactor for certain hydroxylases, including phenylalanine-4-, tyrosine-3-, tryptophan-5-, and glyceryl ether-monooxygenase, as well as nitric oxide synthase (NOS). In this review, the focus will be on the diseases and pathophysiology arising from biopterin dysregulation. In addition to genetic diseases arising from bioperin deficiency ”Atypical phenylketonuria” and ”Dopa-responsive dystonia”, decreased levels of BH4 have also been documented in other neurological diseases presenting phenotypically without hyperphenylalaninemia. As animal models, hph-1 mouse and 6-pyruvoyltetrahydropterin synthase knockout mouse are presented. Intracellular levels of BH4 dictate the rate of NO synthesis by NOSs and even determine the end product, NO, peroxynitrite (OONO^-), or O2^-. Thus NOS-related pathophysiology resulted from biopterin dysregulation should be appreciated.
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