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[恶性贫血患者红细胞与δ-氨基乙酰丙酸孵育后的胆色素原合酶活性及卟啉生物合成]

[Porphobilinogen synthase activity and porphyrin biosynthesis in the erythrocytes after incubation with delta-aminolevulinic acid in pernicious anemia patients].

作者信息

Arsov Ts, Georgiev G

出版信息

Vutr Boles. 1987;26(2):91-5.

PMID:3604204
Abstract

The activity of PBG-S was studied in erythrocytes according the standard method of Berlin and Schaller as well as the biosynthesis of porphyrins after incubation with delta-aminolevulinic acid according to Idel'son in a control group of 40 healthy males and females and 15 patients with pernicious anemia. The activity of porphobilinogen synthase and the level of porphobilinogen were elevated in the patients studied. The total quantity of synthesized porphyrins is increased as regards the control group of healthy on the account of the enhanced biosynthesis of uroporphyrins and coproporphyrins, whereas the quantity of the protoporphyrin synthesized was reduced. The changes established reflect the secondary disordered heme synthesis in the patients with pernicious anemia.

摘要

按照柏林和沙勒的标准方法,在红细胞中研究了胆色素原合酶(PBG-S)的活性;按照伊德尔森的方法,在40名健康男性和女性的对照组以及15名恶性贫血患者中,研究了与δ-氨基乙酰丙酸孵育后卟啉的生物合成。在所研究的患者中,胆色素原合酶的活性和胆色素原的水平升高。由于尿卟啉和粪卟啉的生物合成增强,与健康对照组相比,合成卟啉的总量增加,而合成的原卟啉量减少。所发现的这些变化反映了恶性贫血患者血红素合成的继发性紊乱。

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