Figure 3.

Mitochondrial defects contribute to immune system activation and renal failure. Mitochondrial defects, such as loss of mitochondrial transcription factor (Tfam), leads to cytosolic release of mitochondrial DNA (mtDNA). Cytosolic DNA is recognized by the cyclic guanosine monophosphate–adenosine monophosphate synthase (cGAS)/stimulator of interferon genes (STING) pathway, leading to the activation of nuclear factor-κB (NF-κb), resulting in cytokine release, cell death, influx of immune cells, and, ultimately, renal failure. Abbreviations: OXPHOS, oxidative phosphoryation; TFAM, mitochondrial transcription factor.