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色氨酸吡咯酶、游离血红素的调节与肝性卟啉症。临床及实验性卟啉症加重因素导致的血红素早期消耗。

Tryptophan pyrrolase, the regulatory free haem and hepatic porphyrias. Early depletion of haem by clinical and experimental exacerbators of porphyria.

作者信息

Badawy A A

出版信息

Biochem J. 1978 Jun 15;172(3):487-94. doi: 10.1042/bj1720487.

Abstract
  1. The importance of the early depletion of liver haem in the production of porphyria is discussed and further supporting evidence is presented from experiments with tryptophan pyrrolase, under conditions of exacerbation of experimental porphyria by therapeutic and other agents. 2. In addition to the early depletion of pyrrolase haem by porphyrogens, a further depletion is produced when rats are given a porphyrogen plus an analogue or one of 19 drugs known to exacerbate the human disease. 3. Non-exacerbators of human porphyrias do not cause a further early depletion of pyrrolase haem and it is suggested that this system may be used as a screening test for possible exacerbation of the disease by new and existing drugs. 4. A similar further early depletion of haem is produced by combined administration of lead acetate plus phenobarbitone, thus suggesting that the depletion is a more general phenomenon in experimental porphyria. 5. The relationship between tryptophan pyrrolase and the regulatory free haem is discussed. It is suggested that pyrrolase may play an important role in the regulation of haem biosynthesis.
摘要
  1. 讨论了肝脏血红素早期耗竭在卟啉症产生中的重要性,并在治疗剂和其他药剂加剧实验性卟啉症的条件下,通过色氨酸吡咯酶实验提供了进一步的支持证据。2. 除了卟啉原使吡咯酶血红素早期耗竭外,当给大鼠服用卟啉原加一种类似物或已知会加剧人类疾病的19种药物之一时,会产生进一步的耗竭。3. 人类卟啉症的非加剧剂不会导致吡咯酶血红素进一步早期耗竭,并且表明该系统可用于筛选新药和现有药物可能加剧该疾病的情况。4. 醋酸铅加苯巴比妥联合给药会产生类似的血红素进一步早期耗竭,因此表明这种耗竭在实验性卟啉症中是一种更普遍的现象。5. 讨论了色氨酸吡咯酶与调节性游离血红素之间的关系。有人认为吡咯酶可能在血红素生物合成的调节中起重要作用。

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