Mitochondria are the sole semiautonomous organelles of the human cell and
play a very important role in not only energy production but also in apoptosis,
metabolism and cell signaling. They are also known to be major producers of ROS and
RNS free radicals during ATP production. These free radicals in excessive amount may
damage the mitochondrial DNA as well as proteome resulting in accumulation of
misfolded proteins which may prove deleterious to their functioning and are known to be
involved in disease pathology. To maintain healthy proteome, mitochondria have
developed as quality control machinery in semiautonomous manner, where cellular
proteins such as proteases and heat shock proteins are used for quality control. The
present review discusses various aspects of mitochondrial protein quality control
operating at outer or inner membrane as well as intermembranal space. The various
pathways involved in mitochondrial unfolded protein response have been discussed
along with their implications in cancer and various neurodegenerative diseases.
Keywords: Mitochondria UPR, UPS, protein misfolding, chaperons, protein homeostasis, ATP production.
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