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一名患有线粒体脑肌病和肾功能不全患者各组织中复合物I亚基水平的差异。

Variation in the levels of complex I subunits among tissues in a patient with mitochondrial encephalomyopathy and renal dysfunction.

作者信息

Tanaka M, Nishikimi M, Suzuki H, Ozawa T, Ichiki T, Kobayashi M, Wada Y

机构信息

Department of Biomedical Chemistry, Faculty of Medicine, University of Nagoya, Japan.

出版信息

Biochem Int. 1987 Apr;14(4):735-9.

PMID:3134026
Abstract

Enzymic activity and the levels of immunochemically detectable subunits of NADH-ubiquinone oxidoreductase (Complex I) were measured in the mitochondria from various tissues of a patient with mitochondrial encephalomyopathy and renal dysfunction. Rotenone-sensitive NADH-cytochrome c reductase activity was decreased in all the tissues examined, but the degree of deficiency varied from tissue to tissue. The levels of subunits in Complex I were decreased roughly in parallel with the activity in each tissue. These results indicate that the apparently tissue-specific manifestation of symptoms depends mainly on the levels of subunits in Complex I.

摘要

在一名患有线粒体脑肌病和肾功能障碍患者的各种组织的线粒体中,测定了NADH-泛醌氧化还原酶(复合体I)的酶活性和免疫化学可检测亚基的水平。在所检查的所有组织中,鱼藤酮敏感的NADH-细胞色素c还原酶活性均降低,但缺乏程度因组织而异。复合体I中亚基的水平大致与每个组织中的活性平行降低。这些结果表明,症状明显的组织特异性表现主要取决于复合体I中亚基的水平。

相似文献

1
Variation in the levels of complex I subunits among tissues in a patient with mitochondrial encephalomyopathy and renal dysfunction.一名患有线粒体脑肌病和肾功能不全患者各组织中复合物I亚基水平的差异。
Biochem Int. 1987 Apr;14(4):735-9.
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Disproportionate deficiency of iron-sulfur clusters and subunits of complex I in mitochondrial encephalomyopathy.
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Deficiency of subunits of Complex I and mitochondrial encephalomyopathy.复合体I亚基缺乏与线粒体脑肌病
Ann Neurol. 1988 Mar;23(3):287-94. doi: 10.1002/ana.410230312.
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Deficiency of subunits in heart mitochondrial NADH-ubiquinone oxidoreductase of a patient with mitochondrial encephalomyopathy and cardiomyopathy.
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A reductase/isomerase subunit of mitochondrial NADH:ubiquinone oxidoreductase (complex I) carries an NADPH and is involved in the biogenesis of the complex.线粒体NADH:泛醌氧化还原酶(复合体I)的一种还原酶/异构酶亚基携带一个NADPH,并参与该复合体的生物合成。
J Mol Biol. 1999 Sep 24;292(3):569-80. doi: 10.1006/jmbi.1999.3096.

引用本文的文献

1
Clinical and molecular heterogeneity in patients with mitochondrial encephalomyopathies due to complex I deficiency.由于复合体I缺乏导致的线粒体脑肌病患者的临床和分子异质性。
J Inherit Metab Dis. 1988;11(3):333-6. doi: 10.1007/BF01800387.
2
Deficiency of subunits of complex I or IV in mitochondrial myopathies: immunochemical and immunohistochemical study.线粒体肌病中复合物I或IV亚基的缺乏:免疫化学和免疫组织化学研究
J Inherit Metab Dis. 1987;10(3):284-8. doi: 10.1007/BF01800083.