Medical Research Council Mitochondrial Biology Unit, University of Cambridge, Cambridge, United Kingdom.
Groningen Biomolecular Sciences and Biotechnology Institute, Membrane Enzymology, University of Groningen, Groningen, The Netherlands.
Physiology (Bethesda). 2020 Sep 1;35(5):302-327. doi: 10.1152/physiol.00009.2020.
Members of the mitochondrial carrier family (SLC25) transport a variety of compounds across the inner membrane of mitochondria. These transport steps provide building blocks for the cell and link the pathways of the mitochondrial matrix and cytosol. An increasing number of diseases and pathologies has been associated with their dysfunction. In this review, the molecular basis of these diseases is explained based on our current understanding of their transport mechanism.
线粒体载体家族(SLC25)的成员将各种化合物穿过线粒体的内膜进行运输。这些运输步骤为细胞提供了构建模块,并连接了线粒体基质和细胞质的途径。越来越多的疾病和病理与它们的功能障碍有关。在这篇综述中,根据我们目前对其运输机制的理解,解释了这些疾病的分子基础。