缺陷酵素
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A. PRPP合成酶(PRPP synthetase)：為合成嘌呤過程中第一個步驟的酵素
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狀況一
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PRPP合成酶(synthetase)過度活化(Vmax增加)
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狀況二
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正常情況：產物副回饋抑制作用→嘌呤(purine)會抑制PRPP合成酶
異常情況：負回饋作用被抑制，致使產物過量
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狀況三
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PRPP合成酶的Km值下降、親和力上升，因此酵素對反應物(ribose-5-phosphate)很敏感，意即少量的核糖核酸(ribose 5-phosphate)就能使PRPP 合成酶作用
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B. 次黃嘌呤-鳥糞嘌呤磷酸核糖轉移酶(HGPRTase，hypoxanthine-guanine phosphoribosyltransferase)
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狀況
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HGPRTase部分缺乏，導致salvage pathway出問題，
次黃嘌呤(hypoxanthine)和鳥糞嘌呤(guanine)無法有效率地變成IMP和GMP，因而大量轉變為黃嘌呤(xanthine) →尿酸過多
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結果
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皆會導致PRPP過多→嘌呤(purine)過多→痛風
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遺傳形式
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性聯隱性遺傳疾病(X-linked recessive)
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▼遺傳物質(genetic material)
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Great post, i deal usually with gout sufferers and so that my prefered topic i read more about it, yes there is a lot resources about what cause the rise of uric acid blood level actually there is much information and everything seems clear but frankly until now there isn't a true solution to gout or at least a solution without fatal side effects, in such situation i recommend mostly to any gout sufferer to opt for a healthy lifestyle, good habits and a low purines gout diet, as well to improve their autonomy, personal and professional life quality i recomment often a special gout lotion for Immediate gout pain relief
回覆刪除Waiting your next posts,
Good cheers,