CHAPTER C7 – The hph-1 Mouse
作者： KEITH HYLAND , SIMON J.R. HEALES
DOI: 10.1016/B978-012088382-0/50025-6
关键词: Endocrinology 、 Cofactor 、 Serotonin 、 Phenylalanine 、 Nitric oxide 、 GTP' 、 Tetrahydrobiopterin 、 Internal medicine 、 Enzyme 、 Guanosine triphosphate 、 Biology
摘要: The hph-1 mouse has a deficiency of guanosine triphosphate (GTP) cyclohydrolase that leads to decreased synthesis tetrahydrobiopterin. This is cofactor required for the metabolism phenylalanine and serotonin, catecholamines, nitric oxide. mouse, therefore, provides platform studying pathophysiological effects abnormalities in these areas metabolism. There are no overt signs movement disorder on spontaneous open field behavior but biochemical features suggest good model investigation dominantly inherited GTP oxide deficiency. In cause elevated plasma levels shown be tetrahydrobiopterin, which acts as hydroxylas enzyme. particular, closely mimic those seen cyclohydrolase-deficiency, careful may unravel some intriguing questions remain unanswered this disorder. Overt any absent, it likely specialized testing future can reveal abnormalities. case, suitable pharmacological interventions.
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