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Looking into mitochondrial disease (Mitochondrial biogenesis and…
Looking into mitochondrial disease
ATP supply
Mitochondrial biogenesis and degradation
transcription factors regulation
NRF1
NRF2
peroxisome proliferator‑activated receptor‑γ co‑activator 1α (PGC1α)73,75,76,78.
Energy sensor AMP ‑ activated kinase (AMPK) - upon activation by a lowered ATP:ADP or ATP:AMP ratio, inhibits anabolic pathways and stimulates catabolic pathways.
Mitochondrial fission and/or fusion
Fission
DRP1
MDV1
Fusion
MFN1
MFN2
OPA1
Mitochondrial quality control
Degradation of damaged proteins
Repairing of damages
UPRmt activation
ROS production
Induction of the mitochondrial permeability transition pore (MPTP)
Release of pro-apoptotic factors to the cytosol by induction of mitochondrial outer membrane permeabilization (MOMP)
Activation of the innate immune system by release of damage-associated molecular patterns (DAMPs)
Mitochondrial signalling
Calcium homeostasis
repairing or preventing damage to the organelle
inducing mitochondrial biogenesis
enhancing organelle quality control by stimulating degradation of damaged mitochondria or organelle components
co-opting mitochondrial function to induce cell death
altering mitochondrial signalling pathways or metabolic processes
NAD+ depletion