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铅中毒和急性肝卟啉症中血红素生物合成酶活性的改变。

Alterations in the activity of enzymes of haem biosynthesis in lead poisoning and acute hepatic prophyria.

作者信息

Campbell B C, Brodie M J, Thompson G G, Meredith P A, Moore M R, Goldberg A

出版信息

Clin Sci Mol Med. 1977 Oct;53(4):335-40. doi: 10.1042/cs0530335.

Abstract
  1. The activities of six of the enzymes of haem biosynthesis have been assayed in peripheral blood from patients with lead poisoning, acute intermittent porphyria or hereditary coproprophyria. 2. Compared with normal subjects the lead-poisoned subjects had highly significant depression of delta-aminolaevulinate dehydratase, coproporphyrinogen oxidase and ferrochelatase. 3. Lead-poisoned subjects had highly significant elevation of delta-aminolaevulinate synthase activity. 4. delta-Aminolaevulinate synthase activity was inversely related to the haemoglobin concentration. 5. Increased delta-aminolaevulinate synthase and decreased delta-aminolaevulinate dehydratase activity are also found in acute intermittent porphyria. 6. Increased delta-aminolaevulinate synthase, normal prophobilinogen deaminase and uroporphyrinogen decarboxylase and decreased coproporphyrinogen oxidase are found in both lead poisoning and hereditary coproporphyria. 7. These enzyme changes explain the recognized patterns of porphyrins and prophyrin precurosrs in blood and urine in these conditions.
摘要
  1. 已对铅中毒、急性间歇性卟啉病或遗传性粪卟啉病患者外周血中血红素生物合成的六种酶的活性进行了测定。2. 与正常受试者相比,铅中毒受试者的δ-氨基乙酰丙酸脱水酶、粪卟啉原氧化酶和亚铁螯合酶活性显著降低。3. 铅中毒受试者的δ-氨基乙酰丙酸合酶活性显著升高。4. δ-氨基乙酰丙酸合酶活性与血红蛋白浓度呈负相关。5. 在急性间歇性卟啉病中也发现δ-氨基乙酰丙酸合酶增加和δ-氨基乙酰丙酸脱水酶活性降低。6. 在铅中毒和遗传性粪卟啉病中均发现δ-氨基乙酰丙酸合酶增加、胆色素原脱氨酶和尿卟啉原脱羧酶正常以及粪卟啉原氧化酶降低。7. 这些酶的变化解释了在这些情况下血液和尿液中公认的卟啉和卟啉前体模式。

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