Next Article in Journal
Unusual Manifestation of Live Staphylococcus saprophyticus, Corynebacterium urinapleomorphum, and Helicobacter pylori in the Gallbladder with Cholecystitis
Next Article in Special Issue
Bacillus anthracis Edema Toxin Inhibits Efferocytosis in Human Macrophages and Alters Efferocytic Receptor Signaling
Previous Article in Journal
Bosutinib Inhibits EGFR Activation in Head and Neck Cancer
Previous Article in Special Issue
Bacterial Toxins and Targeted Brain Therapy: New Insights from Cytotoxic Necrotizing Factor 1 (CNF1)
Open AccessCommunication

The Bacterial Protein CNF1 as a Potential Therapeutic Strategy against Mitochondrial Diseases: A Pilot Study

1
Italian Center for Global Health, Istituto Superiore di Sanità, Viale Regina Elena, 299, 00161 Rome, Italy
2
Department of Veterinary Public Health and Food Safety, Istituto Superiore di Sanità, Viale Regina Elena, 299, 00161 Rome, Italy
3
Department of Biosciences, Biotechnologies and Biopharmaceutics, University of Bari, via Orabona, 4, 70124 Bari, Italy
4
Unità di Neurofisiopatologia, Area Neuroscienze, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Largo Agostino Gemelli, 8, 00168 Rome, Italy
*
Author to whom correspondence should be addressed.
Int. J. Mol. Sci. 2018, 19(7), 1825; https://doi.org/10.3390/ijms19071825
Received: 7 June 2018 / Accepted: 18 June 2018 / Published: 21 June 2018
(This article belongs to the Special Issue Bacterial Protein Toxins: Enemies within or Unexpected Friends)
The Escherichia coli protein toxin cytotoxic necrotizing factor 1 (CNF1), which acts on the Rho GTPases that are key regulators of the actin cytoskeleton, is emerging as a potential therapeutic tool against certain neurological diseases characterized by cellular energy homeostasis impairment. In this brief communication, we show explorative results on the toxin’s effect on fibroblasts derived from a patient affected by myoclonic epilepsy with ragged-red fibers (MERRF) that carries a mutation in the m.8344A>G gene of mitochondrial DNA. We found that, in the patient’s cells, besides rescuing the wild-type-like mitochondrial morphology, CNF1 administration is able to trigger a significant increase in cellular content of ATP and of the mitochondrial outer membrane marker Tom20. These results were accompanied by a profound F-actin reorganization in MERRF fibroblasts, which is a typical CNF1-induced effect on cell cytoskeleton. These results point at a possible role of the actin organization in preventing or limiting the cell damage due to mitochondrial impairment and at CNF1 treatment as a possible novel strategy against mitochondrial diseases still without cure. View Full-Text
Keywords: cytotoxic necrotizing factor 1; mitochondrial diseases; myoclonic epilepsy with ragged red fibers syndrome; actin cytoskeleton; adenosine triphosphate cytotoxic necrotizing factor 1; mitochondrial diseases; myoclonic epilepsy with ragged red fibers syndrome; actin cytoskeleton; adenosine triphosphate
Show Figures

Figure 1

MDPI and ACS Style

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

AMA Style

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 Style

Fabbri, Alessia; Travaglione, Sara; Maroccia, Zaira; Guidotti, Marco; Pierri, Ciro L.; Primiano, Guido; Servidei, Serenella; Loizzo, Stefano; Fiorentini, Carla. 2018. "The Bacterial Protein CNF1 as a Potential Therapeutic Strategy against Mitochondrial Diseases: A Pilot Study" Int. J. Mol. Sci. 19, no. 7: 1825. https://doi.org/10.3390/ijms19071825

Find Other Styles
Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Article Access Map by Country/Region

1
Search more from Scilit
 
Search
Back to TopTop