The Bacterial Protein CNF1 as a Potential Therapeutic Strategy against Mitochondrial Diseases: A Pilot Study
Abstract
:1. Introduction
2. Results
2.1. CNF1 Increases the ATP Content and Impacts Mitochondrial Morphology and Mass in Fibroblasts from MA19 Patient Biopsy
2.2. CNF1 Restores, in MA19 Fibroblasts, the Actin Stress Fibers’ Organization Typical of Control Cells
3. Discussion
4. Materials and Methods
4.1. Patients Recruitment and Histochemical Characterization
4.2. Fibroblasts and Treatments
4.3. CNF1 Preparation and Purification
4.4. Fluorescence Microscopy
4.5. Protein Extraction and Western Blot
4.6. Measurement of ATP Content
4.7. Statistical Analysis
Author Contributions
Acknowledgments
Conflicts of Interest
Abbreviations
CNF1 | cytotoxic necrotizing factor 1 |
MERRF | myoclonic epilepsy with ragged-red fibers |
MDs | mitochondrial diseases |
RBF | ragged-blue fibers |
mtDNA | mitochondrial DNA |
Drp1 | dynamin-related protein 1 |
SDH | succinate dehydrogenase |
COX | cytochrome c oxidase |
Tom20 | translocase of the outer mitochondrial membrane |
F-actin | filamentous actin |
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Description | MERRF |
---|---|
age at biopsy (years) | 50 |
ragged-blue fibers (RBF) | +++ |
cytochrome c oxidase (COX) negative RBF | + |
% m.8344A>G mtDNA mutation | muscle: 78; blood: 65 |
% muscle COX activity (normalized for citrate synthase) | 60 |
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Fabbri, A.; Travaglione, S.; Maroccia, Z.; Guidotti, M.; Pierri, C.L.; Primiano, G.; Servidei, S.; Loizzo, S.; Fiorentini, C. The Bacterial Protein CNF1 as a Potential Therapeutic Strategy against Mitochondrial Diseases: A Pilot Study. Int. J. Mol. Sci. 2018, 19, 1825. https://doi.org/10.3390/ijms19071825
Fabbri A, Travaglione S, Maroccia Z, Guidotti M, Pierri CL, Primiano G, Servidei S, Loizzo S, Fiorentini C. The Bacterial Protein CNF1 as a Potential Therapeutic Strategy against Mitochondrial Diseases: A Pilot Study. International Journal of Molecular Sciences. 2018; 19(7):1825. https://doi.org/10.3390/ijms19071825
Chicago/Turabian StyleFabbri, Alessia, Sara Travaglione, Zaira Maroccia, Marco Guidotti, Ciro Leonardo Pierri, Guido Primiano, Serenella Servidei, Stefano Loizzo, and Carla Fiorentini. 2018. "The Bacterial Protein CNF1 as a Potential Therapeutic Strategy against Mitochondrial Diseases: A Pilot Study" International Journal of Molecular Sciences 19, no. 7: 1825. https://doi.org/10.3390/ijms19071825