Depletion of mitochondrial DNA in the skeletal muscle of two cirrhotic patients with severe asthenia

Depletion of mitochondrial DNA in the skeletal muscle of two cirrhotic patients with severe asthenia
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DOI:
10.1016/s0378-1119(01)00815-0
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发表时间:
2002-03-06
期刊:
影响因子:
3.5
通讯作者:
Gadaleta, MN
Gadaleta, MN
中科院分区:
生物学3区
文献类型:
--
作者:
Pesce, V;Cormio, A;Gadaleta, MN

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研究人员对两名肝硬化和严重无力患者骨骼肌中线粒体 DNA (mtDNA) 的定性和定量变化进行了研究。通过长延伸PCR (LX-PCR)发现,在这两名患者中发现了4977个(mtDNA(4977))和7436个(mtDNA(7436)) mtDNA缺失,以及其他mtDNA缺失,而10,422个(mtDNA(10,422)) mtDNA缺失不存在。总而言之,患有严重无力的肝硬化患者的线粒体 DNA 质量变化与年龄匹配的健康个体相当。相反,与对照组相比,两名患者的 mtDNA 含量均显着下降。这种 mtDNA 耗竭可能是由于 mtDNA 与疾病相关的氧化损伤增加所致,这可能会影响线粒体基因组的复制,正如其他氧化应激相关疾病中已经表明的那样。 (C) 2002 Elsevier Science B.V. 保留所有权利。
Qualitative and quantitative alterations of mitochondrial DNA (mtDNA) in the skeletal muscle from two patients with cirrhosis and severe asthenia have been studied. The 4977 by (mtDNA(4977)) and the 7436 by (mtDNA(7436)) mtDNA deletions, as well as other mtDNA deletions, revealed by long extension PCR (LX-PCR), were found in the two patients, whereas the 10,422 by (mtDNA(10,422)) mtDNA deletion was absent. Altogether, the qualitative alterations of mtDNA in cirrhotic patients with severe asthenia were comparable to those of age-matched healthy individuals. The mtDNA content, on the contrary, was substantially decreased in both patients with respect to control. Such mtDNA depletion might be explained by an increased, disease-related, oxidative damage to mtDNA, which probably affects the replication of the mitochondrial genome as already suggested in other oxidative stress-associated diseases. (C) 2002 Elsevier Science B.V. All rights reserved.