ABSTRACT

Mitochondria play a significant role in the cells by metabolizing nutrients and producing energy, ATP as well as participating in various cellular processes including reactive oxygen species (ROS) generation, calcium homeostasis, apoptosis and cell survival. Mitochondrial biogenesis and maintenance are regulated by a numbers of transcription factors/proteins, including PGC-1α, AMPK, and Sirtuins. Proteins quality control is essential for maintaining mitochondrial metabolic homeostasis, which requires tight coordination between the nuclear, cytoplasmic and mitochondrial gene expression machineries to fulfil this complex regulation. Any purtbation of mitochondrial protein homeostasis induces unfolded protein response in mitochondria (UPRmt) which can communicate with ER through the mitochondria-associated membranes (MAMs) to induce metabolic inflammation and contribute to the onset of metabolic syndrome, including obesity, insulin resistance, diabetes, and non-alcoholic fatty liver disease (NAFLD). This chapter presents the recent findings on mitochondrial pathophysiological functions and mainly focuses on the latest breakthroughs highlighting a crucial role of organelle crosstalk in the control of metabolic homeostasis and their association with metabolic disease.