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Buonvicino D.,University of Florence | Formentini L.,Centro Of Biologia Molecular Severo Ochoa | Cipriani G.,University of Florence | Chiarugi A.,University of Florence
Journal of Biological Chemistry | Year: 2013

Background: Excessive activation of enzyme poly(ADP-ribose) polymerase-1 (PARP-1) causes ATP depletion and kills cells. Results: We found that in the absence of glucose PARP-1 triggers an adenylate kinase-dependent increase of ATP. Conclusion: PARP-1 hyperactivation is not invariantly related to ATP loss. Significance: This study adds to the complexity of PARP-1 hyperactivity and energy derangement. © 2013 by The American Society for Biochemistry and Molecular Biology, Inc. Published in the U.S.A.

Gallego-Villar L.,Centro Of Biologia Molecular Severo Ochoa | Perez B.,Centro Of Biologia Molecular Severo Ochoa | Ugarte M.,Centro Of Biologia Molecular Severo Ochoa | Desviat L.R.,Centro Of Biologia Molecular Severo Ochoa | Richard E.,Centro Of Biologia Molecular Severo Ochoa
Biochemical and Biophysical Research Communications | Year: 2014

Propionic acidemia (PA), caused by a deficiency of the mitochondrial biotin dependent enzyme propionyl-CoA carboxylase (PCC) is one of the most frequent organic acidurias in humans. Most PA patients present in the neonatal period with metabolic acidosis and hyperammonemia, developing different neurological symptoms, movement disorders and cardiac complications. There is strong evidence indicating that oxidative damage could be a pathogenic factor in neurodegenerative, mitochondrial and metabolic diseases. Recently, we identified an increase in ROS levels in PA patients-derived fibroblasts. Here, we analyze the capability of seven antioxidants to scavenge ROS production in PA patients' cells. Tiron, trolox, resveratrol and MitoQ significantly reduced ROS content in patients and controls' fibroblasts. In addition, changes in the expression of two antioxidant enzymes, superoxide dismutase and glutathione peroxidase, were observed in PA patients-derived fibroblasts after tiron and resveratrol treatment. Our results in PA cellular models establish the proof of concept of the potential of antioxidants as an adjuvant therapy for PA and pave the way for future assessment of antioxidant strategies in the murine model of PA. © 2014 Elsevier Inc. All rights reserved.

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