Effect of Mitochondrial and Cytosolic FXN Isoform Expression on Mitochondrial Dynamics and Metabolism
Abstract
:1. Introduction
2. Results
2.1. Subcellular Localization of Isoform I and II in Patient OE-MSCs and Fibroblasts
2.2. Mitochondrial Network Analysis in OE-MSCs and FRDA-Derived Fibroblasts Overexpressing Frataxin Isoforms
2.3. Mitochondrial Bioenergetics Analysis in Healthy and FRDA OE-MSCs and Fibroblasts
3. Discussion
4. Materials and Methods
4.1. FRDA-Derived Fibroblasts
4.2. Olfactory Ecto-Mesenchymal Stem Cells
4.3. Isoform Plasmids
4.4. HIV-1-Derived Lentiviral Vectors
4.5. Cell Extraction, Subcellular Fractionation and Western Blot Analysis
4.6. Immunofluorescence Staining
4.7. MiNA Analysis
4.8. Cellular Bioenergetics Analysis
4.9. Data and Statistical Analysis
5. Conclusions
Author Contributions
Funding
Acknowledgments
Conflicts of Interest
Abbreviations
ATP | Adenosine Tri-Phosphate |
BSA | Bovine Serum Albumine |
DMEM | Dulbecco’s Modified Eagle Medium |
DRP1 | Dynamin-related protein 1 |
ECL | Enhanced Chemiluminescence |
EGF | Epidermal Growth Factor |
FBS | Fetal Bovine Serum |
FCCP | Carbonyl cyanide-4-trifluoromethoxyphenylhydrazone |
FXN | Frataxin |
FRDA | Friedreich’s ataxia |
hrFGF2 | Human recombinant Fibroblast Growth Factor 2 |
HRP | Horseradish Peroxidase |
ISCs | Iron Sulfur Clusters |
LC3 | Microtubule-associated proteins 1A/1B light chain 3B |
MFN1 | Mitofusin 1 |
MFN2 | Mitofusin 2 |
MiNA | Mitochondrial Network Analysis |
MTS | Mitochondrial Targeting Sequence |
MPP | Mitochondrial Processing Peptidase |
NEAA | Not-essential Amino Acids |
OCR | Oxygen Consumption Rate |
OE-MSC | Olfactory ecto mesenchymal stem cells |
OPA1 | Optic atrophy protein 1 |
PARK | Parkin |
PBS | Phosphate Buffered Saline |
PFA | Paraformaldehyde |
PINK | PTEN-induced kinase 1 |
RT | Room temperature |
SDS | Sodium Dodecyl Sulfate |
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Agrò, M.; Díaz-Nido, J. Effect of Mitochondrial and Cytosolic FXN Isoform Expression on Mitochondrial Dynamics and Metabolism. Int. J. Mol. Sci. 2020, 21, 8251. https://doi.org/10.3390/ijms21218251
Agrò M, Díaz-Nido J. Effect of Mitochondrial and Cytosolic FXN Isoform Expression on Mitochondrial Dynamics and Metabolism. International Journal of Molecular Sciences. 2020; 21(21):8251. https://doi.org/10.3390/ijms21218251
Chicago/Turabian StyleAgrò, Mauro, and Javier Díaz-Nido. 2020. "Effect of Mitochondrial and Cytosolic FXN Isoform Expression on Mitochondrial Dynamics and Metabolism" International Journal of Molecular Sciences 21, no. 21: 8251. https://doi.org/10.3390/ijms21218251