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犬类周期性造血与嘌呤和嘧啶代谢异常有关。

Canine cyclic hematopoiesis is associated with abnormal purine and pyrimidine metabolism.

作者信息

Osborne W R, Hammond W P, Dale D C

出版信息

J Clin Invest. 1983 May;71(5):1348-55. doi: 10.1172/jci110887.

Abstract

Canine cyclic hematopoiesis is an autosomal recessive disease characterized by regular 11-13-d cycles of the neutrophil, reticulocyte, and platelet counts caused by a defect in regulation of marrow stem cell proliferation. Treatment with lithium abrogates cycling of the cell counts in these grey collie dogs. Aware of the defective lymphopoiesis associated with adenosine deaminase and purine nucleoside phosphorylase deficiencies, we hypothesized that abnormal purine or pyrimidine metabolism might be present in these dogs. Using high pressure liquid chromatography, we measured erythrocyte purine and pyrimidine nucleotide levels and plasma purine and pyrimidine nucleosides and bases in normal and grey collie dogs before and during lithium treatment. During neutropenic periods in the grey collies, erythrocyte ATP, GTP, and UTP levels were significantly elevated. Normal dogs made neutropenic with cyclophosphamide did not show such elevations. Lithium treatment normalized the levels of erythrocyte ATP, GTP, and UTP in the grey collies and eliminated the differences between normal and grey collie nucleotide levels. Plasma thymine levels were markedly increased during neutropenia in the grey collie but were not increased in cyclophosphamide-treated normal dogs. The finding of abnormal concentrations of purine and pyrimidine metabolites in these dogs suggest that a metabolic derangement in purine or pyrimidine metabolism may be the cause of the defective stem cell proliferation in this disease.

摘要

犬循环性造血是一种常染色体隐性疾病,其特征为中性粒细胞、网织红细胞和血小板计数呈现规律的11 - 13天周期,这是由骨髓干细胞增殖调节缺陷所致。用锂治疗可消除这些灰色柯利犬的细胞计数周期性变化。鉴于与腺苷脱氨酶和嘌呤核苷磷酸化酶缺乏相关的淋巴细胞生成缺陷,我们推测这些犬可能存在异常的嘌呤或嘧啶代谢。我们使用高压液相色谱法,测定了正常犬和灰色柯利犬在锂治疗前后红细胞嘌呤和嘧啶核苷酸水平以及血浆嘌呤、嘧啶核苷和碱基水平。在灰色柯利犬的中性粒细胞减少减少期期间,红细胞ATP、GTP和UTP水平显著升高。用环磷酰胺诱导中性粒细胞减少的正常犬未出现此类升高。锂治疗使灰色柯利犬的红细胞ATP、GTP和UTP水平恢复正常,并消除了正常犬和灰色柯利犬核苷酸水平之间的差异。灰色柯利犬中性粒细胞减少期间血浆胸腺嘧啶水平显著升高,但环磷酰胺治疗的正常犬未升高。这些犬中嘌呤和嘧啶代谢产物浓度异常的发现表明,嘌呤或嘧啶代谢的代谢紊乱可能是该疾病中干细胞增殖缺陷的原因。

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