Wojtczak Lech, Zabłocki Krzysztof
Zakład Biochemii, Instytut Biologii Doświadczalnej im. Marcelego Nenckiego, Warszawa.
Postepy Biochem. 2008;54(2):129-41.
In animal cell, mitochondria are the main sites of the synthesis of ATP required for cell functioning and survival. On the other hand, mitochondria play a key role in initiating cell programmed death (apoptosis). In addition, defects in the mitochondrial genome and in the nuclear genome encoding mitochondrial proteins may result in malfunctioning of these organelles and, as result, in diseases of the whole organism. This article contains basic information on the functioning of oxidative phosphorylation and on mitochondrial production of reactive oxygen species. It also describes initiation of apoptosis at the mitochondrial level. Finally, it briefly presents some most common genetic defects responsible for "mitochondrial diseases".
在动物细胞中,线粒体是细胞功能和生存所需ATP合成的主要场所。另一方面,线粒体在启动细胞程序性死亡(凋亡)中起关键作用。此外,线粒体基因组以及编码线粒体蛋白的核基因组中的缺陷可能导致这些细胞器功能异常,进而导致整个生物体患病。本文包含有关氧化磷酸化功能以及线粒体活性氧生成的基本信息。它还描述了线粒体水平上凋亡的启动。最后,简要介绍了一些导致“线粒体疾病”的最常见遗传缺陷。