Hewton Keeley G, Johal Amritpal S, Parker Seth J
Department of Biochemistry & Molecular Biology, University of British Columbia, Vancouver, BC V6T 1Z4, Canada.
British Columbia Children's Hospital Research Institute, Vancouver, BC V6H 0B3, Canada.
Metabolites. 2021 Feb 16;11(2):112. doi: 10.3390/metabo11020112.
Mitochondria are central organelles that coordinate a vast array of metabolic and biologic functions important for cellular health. Amino acids are intricately linked to the bioenergetic, biosynthetic, and homeostatic function of the mitochondrion and require specific transporters to facilitate their import, export, and exchange across the inner mitochondrial membrane. Here we review key cellular metabolic outputs of eukaryotic mitochondrial amino acid metabolism and discuss both known and unknown transporters involved. Furthermore, we discuss how utilization of compartmentalized amino acid metabolism functions in disease and physiological contexts. We examine how improved methods to study mitochondrial metabolism, define organelle metabolite composition, and visualize cellular gradients allow for a more comprehensive understanding of how transporters facilitate compartmentalized metabolism.
线粒体是核心细胞器，协调着对细胞健康至关重要的大量代谢和生物学功能。氨基酸与线粒体的生物能量、生物合成及稳态功能紧密相连，需要特定转运蛋白来促进其在线粒体内膜上的输入、输出及交换。在此，我们综述真核细胞线粒体氨基酸代谢的关键细胞代谢产物，并讨论已知和未知的相关转运蛋白。此外，我们还讨论了在疾病和生理背景下，分区化氨基酸代谢功能的利用情况。我们研究了改进的线粒体代谢研究方法、细胞器代谢物组成的定义以及细胞梯度的可视化如何有助于更全面地理解转运蛋白如何促进分区化代谢。
