Mitochondrial ATP-binding cassette proteins
Mitochondrial ATP-binding cassette proteins
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DOI：
10.1016/j.trsl.2007.03.002
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发表时间：
2007-08-01
影响因子：
7.8
通讯作者：
Ardehali, Hossein
中科院分区：
文献类型：
--
作者：
Burke, Michael A.;Ardehali, Hossein
ATP结合盒（ABC）蛋白家族是生物学中最大且最多样化的家族之一。该家族的成员是在所有生物体和从质膜到细胞内细胞器如高尔基体、溶酶体、过氧化物酶体、内质网和线粒体的所有生物膜中发现的跨膜蛋白。这些蛋白质在细菌中非常丰富，并且考虑到线粒体普遍接受的来自α-变形菌的起源，假设线粒体也含有这些蛋白质是合乎逻辑的。然而，令人惊讶的是，线粒体的ABC蛋白质很少，而且它们与细菌的ABC蛋白质不同。尽管线粒体ABC蛋白相对较少，但据信在非常不同的物种（包括酵母、高等植物、小鼠和人类）的细胞内稳态中发挥非常重要的作用。酵母蛋白atm 1 p在Fe/S簇向胞质溶胶的运输中起着关键作用，类似的功能已被归因于同源的人类蛋白MTABC 3和ABC 7。另一种酵母蛋白Mdl 1 p是ATM 1的高拷贝抑制因子，调节细胞对氧化应激的抗性，并可能参与跨线粒体膜的肽转运。人类蛋白质mABC 1最近已被确定参与保护心肌细胞免受氧化应激。尽管数量较少，但线粒体ABC蛋白复杂地参与线粒体和细胞内稳态，并且可能是细胞存活的重要介质。在这篇综述中，我们将讨论这些线粒体ABC蛋白的结构，功能，生理和病理生理。
The family of ATP-binding cassette (ABC) proteins is among the largest and most diverse in biology. Members of this family are transmembrane proteins found in all organisms and all biologic membranes from the plasma membrane to intracellular organelles such as the Golgi apparatus, lysosomes, peroxisomes, endoplasmic reticulum, and mitochondria. These proteins are very abundant in bacteria, and given the generally accepted origin of mitochondria from an alpha-proteobacterium, it is logical to assume the mitochondria would also contain these proteins. Mitochondria, however, have surprisingly few ABC proteins and they are dissimilar from those of bacteria. Despite their relative paucity, mitochondrial ABC proteins are believed to play a very important role in cellular homeostasis across very diverse species, including yeast, higher plants, mice, and humans. The yeast protein atm1p plays a critical role in the transport of Fe/S clusters to the cytosol, and a similar function has been attributed to the homologous human proteins MTABC3 and ABC7. Another yeast protein Mdl1p is a high copy suppressor of ATM1, and regulates cellular resistance to oxidative stress and may be involved in peptide transport across the mitochondrial membrane. The human protein mABC1 has recently been identified to be involved in protection of myocardial cells against oxidative stress. Despite their low numbers, mitochondrial ABC proteins are intricately involved in mitochondrial and cellular homeostasis and may be important mediators of cell survival. In this review, we will discuss the structure, function, physiology, and pathophysiology of these mitochondrial ABC proteins.