Mitochondrial transport proteins: Highly dynamic integral membrane proteins required for the translocation of metabolites and cofactors, key to the function of the mitochondrion

线粒体的外膜是可渗透的小分子，因为它包含大孔，但内膜，形成嵴，是紧密密封的。大量不同的分子需要穿过内膜以连接细胞溶质和线粒体的生化途径以及用于细胞过程。在这些化合物中，酮酸来源于糖，脂肪酸来源于脂肪，它们在线粒体内氧化产生细胞燃料ATP。来自蛋白质的氨基酸也进入线粒体，在那里它们被分解，相互转化或用于蛋白质合成。线粒体基质中需要许多维生素，因为它们作为许多线粒体酶的辅因子。最后，线粒体DNA复制和转录所需的核苷酸也需要穿过内膜。超过60种不同的转运蛋白存在于线粒体内膜中，以促进这些化合物的转运。它们属于不同的蛋白质家族，如ABC转运蛋白家族、线粒体丙酮酸载体家族和线粒体载体家族。其中许多机构的职能尚未确定。我们有兴趣研究它们在细胞代谢和人体生理学中的作用。我们还想确定它们是如何工作的，以便确定与其功能障碍相关的大量疾病的分子原因。

The outer membrane of mitochondria is permeable for small molecules as it contains large pores, but the inner membrane, which forms cristae, is tightly sealed. A large number of different molecules need to traverse the inner membrane to link the biochemical pathways of the cytosol and mitochondria and for cellular processes. Among these compounds are keto acids derived from sugars and fatty acids derived from fat, which are oxidised inside mitochondria to generate the cellular fuel ATP. Amino acids, derived from protein, also enter mitochondria, where they are broken down, interconverted, or used in protein synthesis. Many vitamins are required in the mitochondrial matrix as they act as co-factors for many mitochondrial enzymes. Finally, nucleotides, which are required for replication and transcription of mitochondrial DNA, also need to traverse the inner membrane. More than 60 different transport proteins are present in the mitochondrial inner membrane to facilitate the translocation of these compounds. They belong to different protein families, such as the ABC transporter family, the mitochondrial pyruvate carrier family, and the mitochondrial carrier family. The function of many of them has not been established. We are interested in studying their role in cellular metabolism and human physiology. We would also like to establish how they work in order to identify the molecular cause for the large number of diseases that are associated with their dysfunction.