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Protein quality control in mitochondria is essential for proper protein homeostasis, which maintains the mitochondrial function well and prevents the proteotoxic stress leading to cellular pathology.Most of mitochondrial proteins are located in the matrix, including the Tricarboxylic Acid Cycle enzymes, enzymes required for-oxidation of fatty acids.Mitochondrial matrix contains three ATP-dependent proteases-the Lon protease, caseinolytic peptidase XP (ClpXP) and matrix-oriented AAA (m-AAA).