Protein transport along the mitochondria carrier pathway (P13)

具有内部靶向信号的多跨内膜蛋白通过载体输入途径进入线粒体。TOM70是这类前体蛋白的受体，并引导它们进入TOM复合物。TIM22复合物利用（）作为驱动力介导内膜插入。只有一大堆的底物已经被实验定义，并且对输运的机制也知之甚少。在这里，我们将对人类和酵母载体转位的功能进行分析。我们的目标是定义载体途径的线粒体亚定位，以了解从TOM到TIM22复合物的前体转移。鉴定TIM22复合物与人类疾病之间的联系使我们能够解决代谢活跃的人类细胞中载体途径的功能障碍。中心目标是确定AGK和TIM22功能障碍的分子病理学。

Multispanning inner membrane proteins with internal targeting signals are transported into mitochondria by the carrier import pathway. TOM70 is the receptor for this class of precursor proteins and directs them to the TOM complex. The TIM22 complex mediates inner membrane insertion utilizing the  as driving force. Only a hand full of substrates has been experimentally defined and the mechanism of transport is only poorly understood. Here we will carry out analyses on the function of the human and yeast carrier translocases. We aim to define the mitochondrial sublocalization of the carrier pathway to understand transfer of precursors from TOM to the TIM22 complex. The identification of links between the TIM22 complex and human diseases has led us to address the dysfunctions of the carrier pathway in metabolically active human cells. A central goal is to define the molecular pathology of AGK and TIM22 dysfunction.