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題名 | Disease and Pathophysiology Arising from Biopterin Dysregulation=生物蝶呤失調所引起的疾病和病理變化 |
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作 者 | Hattori,Yoshiyuki; | 書刊名 | Journal of Medical Sciences |
卷期 | 22:3 2002.06[民91.06] |
頁次 | 頁95-99 |
分類號 | 415.121 |
關鍵詞 | 生物蝶呤失調; 病理; Dysregulation; Pathophysiology; Tetrahydrobiopterin; |
語文 | 英文(English) |
英文摘要 | Tetrahydrobiopterin (BH4) is required as cofactor for certain hydroxylases, including phenylala-nine-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”, de- creased 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. |
本系統之摘要資訊系依該期刊論文摘要之資訊為主。