Mitochondrial unfolded protein response
Pathway4 papers3 findingsGO 0034514
In human dermal fibroblasts, the Tardbp G376D mutation activates the mitochondrial unfolded protein response. In worms, usp-48 mediates the response, whereas unsaturated fatty acids have no effect on it.
- Model organisms2
- Cells1
Interventions
2Unsaturated fatty acids
no changein worms1
1 study
Unsaturated fatty acids
no changein worms1
Unsaturated fatty acids have no effect on mitochondrial unfolded protein response in worms.
Model organisms
Lipid metabolism regulators and snoRNPs control mitochondrial surveillance and mitophagy · GeroScience · 26 Sep 2026
Tardbp G376D mutation
upin human cells1
1 study
Tardbp G376D mutation
upin human cells1
Tardbp G376D mutation activates mitochondrial unfolded protein response in human cells.
“Furthermore, TDP-43 G376D induces sustained activation of the mitochondrial Unfolded Protein Response (UPR mt ), consistent with persistent mitochondrial stress”
Cellsdermal fibroblast
Mitochondrial Quality Control Impairment Is a Hallmark of TDP-43G376D ALS Patient-Derived Fibroblasts · Antioxidants (Basel, Switzerland) · 22 Aug 2026
Upstream
1usp-48
mediatesin worms1
1 study
usp-48
mediatesin worms1
usp-48 mediates mitochondrial unfolded protein response in worms.
Model organisms
USP-48 modulates mitochondrial stress and enables longevity in low-insulin worms · Cell reports · 30 Sep 2026
Latest
4USP-48 modulates mitochondrial stress and enables longevity in low-insulin worms
A genome-wide screen in roundworms links histone H2B deubiquitination by USP-48 to mitochondrial stress signaling and extended lifespan.
Lipid metabolism regulators and snoRNPs control mitochondrial surveillance and mitophagy
Supplementing unsaturated fatty acids enhanced mitochondrial stress responses and pathogen defense without altering the unfolded protein response.