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犬红细胞丙酮酸激酶。II。与巴仙吉犬溶血性贫血相关的异常酶的特性。

Canine erythrocyte pyruvate kinase. II. Properties of abnormal enzyme associated with hemolytic anemia in the Basenji dog.

作者信息

Standerfer R J, Rittenberg M B, Chern C J, Templeton J W, Black J A

出版信息

Biochem Genet. 1975 Jun;13(5-6):341-51. doi: 10.1007/BF00485819.

DOI:10.1007/BF00485819
PMID:810133
Abstract

We have compared the solubility, kinetic, immunological, and electrophoretic properties of erythrocyte pyruvate kinase from normal dogs and Basenji dogs with congenital hemolytic anemia due to pyruvate kinase deficiency. Differences can be detected between the two enzymes by all methods. The enzyme from the affected animals has a greater solubility in ammonium sulfate. It has a lower Km for phosphoenolpyruvate, while the Km for ADP is increased. This enzyme is not inhibited by ATP or activated by fructose 1,6-diphosphate. The enzyme from the affected animals has none of the allosteric properties characteristic of the normal canine enzyme. No difference can be detected by enzyme inactivation with rabbit antiserum against human erythrocyte enzyme, but a slight spur is observed on comparison of the two enzymes by Ouchterlony immunodiffusion. The enzymes also differ in their electrophoretic mobilities on starch gel electrophoresis.

摘要

我们比较了正常犬和因丙酮酸激酶缺乏而患有先天性溶血性贫血的巴仙吉犬红细胞丙酮酸激酶的溶解性、动力学、免疫学和电泳特性。通过所有方法都能检测到这两种酶之间的差异。患病动物的酶在硫酸铵中的溶解度更高。它对磷酸烯醇丙酮酸的米氏常数较低,而对二磷酸腺苷的米氏常数增加。这种酶不受三磷酸腺苷抑制,也不被1,6-二磷酸果糖激活。患病动物的酶没有正常犬类酶所特有的别构性质。用兔抗人红细胞酶血清进行酶失活处理时,未检测到差异,但在双向免疫扩散法比较这两种酶时,观察到了轻微的沉淀线。这两种酶在淀粉凝胶电泳中的电泳迁移率也有所不同。

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引用本文的文献

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Inherited metabolic disease in laboratory animals: a review.实验动物中的遗传性代谢疾病:综述
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Postnatal changes in canine erythrocyte pyruvate kinase isozymes.犬红细胞丙酮酸激酶同工酶的产后变化
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3
Hemolytic anemia due to pyruvate kinase deficiency: characterization of the enzymatic activity from eight patients.丙酮酸激酶缺乏所致的溶血性贫血:8例患者酶活性特征分析

本文引用的文献

1
PYRUVATE KINASE: MOLECULAR DIFFERENCES BETWEEN HUMAN RED CELL AND LEUKOCYTE ENZYME.丙酮酸激酶:人类红细胞与白细胞酶的分子差异
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[Erythrocyte isolation from blood with cotton].[用棉花从血液中分离红细胞]
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Familial anemia in the Basenji dog.巴仙吉犬的家族性贫血
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4
Isolation and characterization of a Saccharomyces cerevisiae mutant deficient in pyruvate kinase activity.一株丙酮酸激酶活性缺陷型酿酒酵母突变体的分离与鉴定
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Molecular pathology of human haemoglobin.人类血红蛋白的分子病理学
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5
Evidence for molecular alteration of pyruvate kinase as a consequence of erythrocyte aging.
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Intraerythrocytic adaptation to anemia.红细胞内对贫血的适应性
N Engl J Med. 1970 Jul 23;283(4):165-9. doi: 10.1056/NEJM197007232830402.
7
A new variant of red blood cell pyruvate kinase deficiency.红细胞丙酮酸激酶缺乏症的一种新变体。
Biochim Biophys Acta. 1972 Feb 28;258(2):685-7. doi: 10.1016/0005-2744(72)90262-8.
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Genetic disorders of red cell metabolism.红细胞代谢的遗传性疾病。
Med Clin North Am. 1969 Jul;53(4):813-26.
9
Anomalous pyruvate kinase deficiency in the Basenji dog.巴仙吉犬丙酮酸激酶异常缺乏症
Am J Vet Res. 1974 Dec;35(12):1541-3.
10
Control of glycolysis in the human red blood cell.人体红细胞中糖酵解的调控
J Biol Chem. 1966 Nov 10;241(21):4848-54.