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缺铁性贫血患者红细胞中的卟啉生物合成

Porphyrin biosynthesis in the erythrocytes of patients with sideropenic anaemias.

作者信息

Gutniak O, Kopéc M, Nieczaj J

出版信息

J Clin Pathol. 1971 May;24(4):336-42. doi: 10.1136/jcp.24.4.336.

Abstract

The ability of erythrocytes to synthesize porphyrins from delta-aminolaevulic acid was compared in healthy people, patients with true iron-deficiency anaemia, and anaemic patients with rheumatoid arthritis. Porphyrin synthesis was found to be impaired in true iron deficiency but not in rheumatoid arthritis cases showing a similar degree of anaemia. The delta-aminolaevulic acid dehydratase activity and the rate of porphobilinogen synthesis did not differ in the three groups studied. The most probable mechanism of impairment of porphyrin synthesis in the anaemia of true iron deficiency seems to be a partial block in the conversion of porphobilinogen into porphyrins. High endogenous and exogenous protoporphyrin concentration did not affect the synthesis of porphyrins.

摘要

对健康人、真性缺铁性贫血患者以及类风湿性关节炎贫血患者的红细胞从δ-氨基乙酰丙酸合成卟啉的能力进行了比较。研究发现,真性缺铁时卟啉合成受损,但在贫血程度相似的类风湿性关节炎病例中未受损。在所研究的三组中,δ-氨基乙酰丙酸脱水酶活性和胆色素原合成速率并无差异。真性缺铁性贫血中卟啉合成受损的最可能机制似乎是胆色素原转化为卟啉的过程存在部分阻滞。内源性和外源性原卟啉的高浓度并未影响卟啉的合成。

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本文引用的文献

2
SIDEROBLASTIC ANAEMIA IN MAN: OBSERVATIONS ON SEVENTY CASES.人类铁粒幼细胞性贫血:70例观察报告
Br J Haematol. 1965 Jan;11:59-69. doi: 10.1111/j.1365-2141.1965.tb00085.x.
4
SIDEROBLASTS AND SIDEROBLASTIC ANAEMIA.环形铁粒幼细胞及铁粒幼细胞性贫血
Br J Haematol. 1965 Jan;11:41-8. doi: 10.1111/j.1365-2141.1965.tb00082.x.

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