Molecules and Cells

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Fig. 1. At the protein level, DNA repair proteins (mitochondrial DNA polymerase gamma) repair mitochondrial DNA; Chaperones import nuclear-encoded mitochondrial proteins into the mitochondria and correctly fold misfolded proteins in the matrix; Mitochondrial outer membrane proteins are ubiquitinated by MARCH5 and extracted by P97 to be degraded by the proteasome; Proteases in the mitochondrial matrix and inter membrane space degrade damaged proteins; Antioxidants reduce reactive oxygen species to protect mitochondria. At the organelle level, mitochondria biogenesis occurs upon specific signaling; the mitochondrial network changes mitochondrial shape through fusion and fission to accommodate metabolic requirement; Mitofusin 1/2 and OPA1 mediate fusion in the mitochondrial outer and inner membrane, respectively. MitoPLD converts cardiolipin to phosphatic acid inducing the fusion of mitochondria. When mitochondrial fission occurs, Drp1 binds to its receptors (MFF, MID49, MID51 and FIS1) to form Drp1 oligomers and tightens mitochondria to divide it. Also, increase of short form OPA1 induces mitochondrial fission. Damaged mitochondria are recognized by autophagosome, isolated and then fused with the lysosome to form an autolysosome and finally degraded.
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