From: Emerging roles and regulation of MiT/TFE transcriptional factors
Core regulatory molecules or complex | Effect on mitochondrial | Related disease model | Reference |
---|---|---|---|
Mitochondrial transcription factor A (TFAM) | Controls mtDNA copy number | Lysosomal storage disorders, sphingolipidoses | [46] |
Peroxisome proliferator coactivator-1 alpha (PGC1α) | Induces mitochondrial biogenesis, mitochondrial remodeling, respiration, gluconeogenesis and glucose transport, fatty acid oxidation, peroxisomal remodeling, and detoxification of reative oxygen species | Muscle wasting myopathies; Cardiac ischemia-reperfusion injury; Parkinson’s disease; Huntington’s disease; melanoma; obesity; Retinal pigment epithelium (RPE)-associated retinal degeneration; Non-alcoholic fatty liver disease (NAFLD) | |
Mitochondrial respiratory chain complex I | Initial and rate limiting enzyme in electron transfer chain | Parkinson’s disease (PD) | [50] |
Mitochondrial respiratory chain complex II | Junction between oxidative phosphorylation and electron transport | Diabetes, obesity, and metabolic syndrome | [41] |
Dynamin-related protein 1 (Drp1) | Key regulator of mitochondrial fission | Lethal dilated cardiomyopathy | [49] |
PEN-induced putative kinase 1 (PINK1) | Recruits parkin resulting in ubiquitination of mitochondrial proteins | Parkinson’s disease (PD) | |
GCN5-like Protein 1 (GCN5L1) | A putative nutrient-sensing regulator, controls mitochondrial removal by autophagy | Fatty liver, Type 2 diabetes | [51] |
STIP1 homology and U-Box containing protein 1 (STUB1) | Promotes ubiquitin-mediated protein degradation | Neurodegenerative diseases | [48] |