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UPRmt activation improves pathological alterations in cellular models of mitochondrial diseases
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UPRmt activation improves pathological alterations in cellular models of mitochondrial diseases
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UPRmt activation improves pathological alterations in cellular models of mitochondrial diseases
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Journal Article
UPRmt activation improves pathological alterations in cellular models of mitochondrial diseases
2022
Overview
Background
Mitochondrial diseases represent one of the most common groups of genetic diseases. With a prevalence greater than 1 in 5000 adults, such diseases still lack effective treatment. Current therapies are purely palliative and, in most cases, insufficient. Novel approaches to compensate and, if possible, revert mitochondrial dysfunction must be developed.
Results
In this study, we tackled the issue using as a model fibroblasts from a patient bearing a mutation in the
GFM1
gene, which is involved in mitochondrial protein synthesis. Mutant
GFM1
fibroblasts could not survive in galactose restrictive medium for more than 3 days, making them the perfect screening platform to test several compounds. Tetracycline enabled mutant
GFM1
fibroblasts survival under nutritional stress. Here we demonstrate that tetracycline upregulates the mitochondrial Unfolded Protein Response (UPR
mt
), a compensatory pathway regulating mitochondrial proteostasis. We additionally report that activation of UPR
mt
improves mutant
GFM1
cellular bioenergetics and partially restores mitochondrial protein expression.
Conclusions
Overall, we provide compelling evidence to propose the activation of intrinsic cellular compensatory mechanisms as promising therapeutic strategy for mitochondrial diseases.
