Research Topics
Genomes and Genes | Paolo BernardiSummaryAffiliation: University of Padova Country: Italy Publications
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Publications
EBEC 2012--an energetic time in FreiburgPaolo Bernardi
Department of Biomedical Sciences, University of Padova, Padova, Italy
EMBO Rep 14:7-9. 2013..The conference is a biannual event that brings together researchers from across the globe to discuss progress in this diverse and challenging field...
The permeability transition pore as a Ca(2+) release channel: new answers to an old questionPaolo Bernardi
Department of Biomedical Sciences and CNR Institute of Neuroscience, University of Padova, Padova, Italy
Cell Calcium 52:22-7. 2012..We also address the relevance of a mitochondrial Ca(2+) release channel recently discovered in Drosophila melanogaster, which possesses intermediate features between the permeability transition pore of yeast and mammals...
The mitochondrial permeability transition from in vitro artifact to disease targetPaolo Bernardi
Department of Biomedical Sciences and CNR Institute of Neurosciences, University of Padova, Italy
FEBS J 273:2077-99. 2006..Finally, we discuss evidence indicating that the permeability transition pore plays a role in pathophysiology, with specific emphasis on in vivo models of disease...
Dysfunction of mitochondria and sarcoplasmic reticulum in the pathogenesis of collagen VI muscular dystrophiesPaolo Bernardi
Department of Biomedical Sciences, University of Padova, Viale Guiseppe Colombo, Padova, Italy
Ann N Y Acad Sci 1147:303-11. 2008....
Genetic ablation of cyclophilin D rescues mitochondrial defects and prevents muscle apoptosis in collagen VI myopathic miceElena Palma
Department of Biomedical Sciences, University of Padova, 35121 Padova, Italy
Hum Mol Genet 18:2024-31. 2009....
The translocator protein (peripheral benzodiazepine receptor) mediates rat-selective activation of the mitochondrial permeability transition by norbormideAlessandra Zulian
C N R Institute of Neurosciences at the Department of Biomedical Sciences, University of Padova, Italy
Biochim Biophys Acta 1807:1600-5. 2011..Thus, species-specificity of NRB toward the rat PT depends on subtle differences in the structure of TSPO or of TSPO-associated proteins affecting its substrate specificity...
Properties of the permeability transition pore in mitochondria devoid of Cyclophilin DEmy Basso
Department of Biomedical Sciences and Consiglio Nazionale delle Ricerche Institute of Neuroscience, University of Padova, Viale Giuseppe Colombo 3, I 35121 Padova, Italy
J Biol Chem 280:18558-61. 2005..These results have major implications for our current understanding of the PTP and its modulation in vitro and in vivo...
Phosphate is essential for inhibition of the mitochondrial permeability transition pore by cyclosporin A and by cyclophilin D ablationEmy Basso
Department of Biomedical Sciences and Consiglio Nazionale delle Ricerche Institute of Neuroscience, University of Padova, Viale Giuseppe Colombo 3, I 35121 Padova, Italy
J Biol Chem 283:26307-11. 2008..These findings call for a reassessment of conclusions on the role of the PTP in cell death that are based on the effects of CsA or of CyP-D ablation...
Modulation of mitochondrial K(+) permeability and reactive oxygen species production by the p13 protein of human T-cell leukemia virus type 1Micol Silic-Benussi
Department of Oncology and Surgical Sciences, University of Padova, I 35128 Padova, Italy
Biochim Biophys Acta 1787:947-54. 2009..The downstream consequences of p13-induced mitochondrial K(+) permeability are likely to have an important influence on the redox state and turnover of HTLV-1-infected cells...
Cyclophilin D in mitochondrial pathophysiologyValentina Giorgio
Department of Biomedical Sciences and CNR Institute of Neuroscience, University of Padova, Italy
Biochim Biophys Acta 1797:1113-8. 2010....
Cyclophilin D modulates mitochondrial F0F1-ATP synthase by interacting with the lateral stalk of the complexValentina Giorgio
Department of Biomedical Sciences and the Consiglio Nazionale delle Ricerche Institute of Neuroscience, University of Padova, I 35121 Padova, Italy
J Biol Chem 284:33982-8. 2009..These findings demonstrate that CyPD association to the lateral stalk of ATP synthase modulates the activity of the complex...
The mitochondrial permeability transition from yeast to mammalsLuca Azzolin
Department of Biomedical Sciences, CNR Institute of Neuroscience, University of Padova, Padova, Italy
FEBS Lett 584:2504-9. 2010..Biochim. Biophys. Acta 1787, 1486-1491] suggest that the mitochondrial permeability transition has been conserved during evolution...
Autophagy is defective in collagen VI muscular dystrophies, and its reactivation rescues myofiber degenerationPaolo Grumati
Department of Histology, Microbiology and Medical Biotechnology, University of Padova, Padova, Italy
Nat Med 16:1313-20. 2010..These findings indicate that defective activation of the autophagic machinery is pathogenic in some congenital muscular dystrophies...
Properties of the permeability transition in VDAC1(-/-) mitochondriaAlexandra Krauskopf
Department of Biomedical Sciences, University of Padova, Viale Giuseppe Colombo 3, I 35121 Padova, Italy
Biochim Biophys Acta 1757:590-5. 2006..The labeled protein could be separated from all VDAC isoforms. While these results do not allow to exclude that VDAC is part of the PTP, they suggest that VDAC is not the target for PTP inhibition by Ro 68-3400...
Regulation of the inner membrane mitochondrial permeability transition by the outer membrane translocator protein (peripheral benzodiazepine receptor)Justina Sileikyte
Department of Biomedical Sciences, Consiglio Nazionale delle Ricerche Institute of Neuroscience, University of Padova, I 35121 Padova, Italy
J Biol Chem 286:1046-53. 2011..Thus, the permeability transition is an inner membrane event that is regulated by the outer membrane through specific interactions with TSPO...
Switch from inhibition to activation of the mitochondrial permeability transition during hematoporphyrin-mediated photooxidative stress. Unmasking pore-regulating external thiolsValeria Petronilli
C N R Institute of Neurosciences at the Department of Biomedical Sciences, University of Padova, Italy
Biochim Biophys Acta 1787:897-904. 2009..These findings shed new light on PTP modulation by oxidative stress...
Mitochondrial Ca2+ transport and permeability transition in zebrafish (Danio rerio)Luca Azzolin
Department of Biomedical Sciences and CNR Institute of Neuroscience, University of Padua, Italy
Biochim Biophys Acta 1797:1775-9. 2010..Our results represent a necessary step toward the use of zebrafish for the screening and validation of PTP inhibitors of potential use in human diseases, as recently shown for collagen VI muscular dystrophy [Telfer et al., 2010]...
Activation of mitochondrial ERK protects cancer cells from death through inhibition of the permeability transitionAndrea Rasola
Department of Biomedical Sciences, National Research Council Institute of Neuroscience and Venetian Institute of Molecular Medicine, University of Padua, 35121 Padua, Italy
Proc Natl Acad Sci U S A 107:726-31. 2010..Thus, in tumor cells mitochondrial ERK activation desensitizes the PTP through a signaling axis that involves GSK-3 and cyclophilin D, a finding that provides a mechanistic basis for increased resistance to apoptosis of neoplastic cells...
The mitochondrial permeability transition pore and cyclophilin D in cardioprotectionFabio Di Lisa
Department of Biomedical Sciences and CNR Institute of Neuroscience, University of Padova, 35121 Padova, Italy
Biochim Biophys Acta 1813:1316-22. 2011..This article is part of a Special Issue entitled: Mitochondria and Cardioprotection...
A CaPful of mechanisms regulating the mitochondrial permeability transitionFabio Di Lisa
Department of Biomedical Sciences and CNR Institute of Neuroscience, University of Padova, Padova, Italy
J Mol Cell Cardiol 46:775-80. 2009....
The mitochondrial effects of small organic ligands of BCL-2: sensitization of BCL-2-overexpressing cells to apoptosis by a pyrimidine-2,4,6-trione derivativeEva Milanesi
Department of Biomedical Sciences and Consiglio Nazionale delle Ricerche Institute of Neurosciences, University of Padova, Viale Giuseppe Colombo 3, I 35121 Padua, Italy
J Biol Chem 281:10066-72. 2006....
Hexokinase II detachment from mitochondria triggers apoptosis through the permeability transition pore independent of voltage-dependent anion channelsFederica Chiara
CNR Institute of Neuroscience and Department of Biomedical Sciences, University of Padova, Padova, Italy
PLoS ONE 3:e1852. 2008..These findings have profound implications for our understanding of the pathways of outer mitochondrial membrane permeabilization and their inactivation in tumors...
Antamanide, a derivative of Amanita phalloides, is a novel inhibitor of the mitochondrial permeability transition poreLuca Azzolin
Department of Biomedical Sciences University of Padova and CNR Institute of Neuroscience, Padova, Italy
PLoS ONE 6:e16280. 2011..Our findings have implications for the comprehension of cyclophilin D activity on the permeability transition pore and for the development of novel pore-targeting drugs exploitable as cell death inhibitors...
Mitochondrial permeability transition in Ca(2+)-dependent apoptosis and necrosisAndrea Rasola
Department of Biomedical Sciences and CNR Institute of Neuroscience, University of Padova, Italy
Cell Calcium 50:222-33. 2011....
Altered threshold of the mitochondrial permeability transition pore in Ullrich congenital muscular dystrophyAlessia Angelin
Department of Biomedical Sciences and CNR Institute of Neuroscience, University of Padova, Italy
Biochim Biophys Acta 1777:893-6. 2008....
Mitochondrial dysfunction and apoptosis in myopathic mice with collagen VI deficiencyWilliam A Irwin
Department of Histology, University of Padova, 35121 Padova, Italy
Nat Genet 35:367-71. 2003..These findings indicate that collagen VI myopathies have an unexpected mitochondrial pathogenesis that could be exploited for therapeutic intervention...
Arachidonic acid released by phospholipase A(2) activation triggers Ca(2+)-dependent apoptosis through the mitochondrial pathwayDaniele Penzo
Department of Biomedical Sciences, Consiglio Nazionale delle Ricerche Institute of Neuroscience at the University of Padova, Viale Giuseppe Colombo 3, I-35121 Padova, Italy
J Biol Chem 279:25219-25. 2004..These findings identify arachidonic acid as the causal link between A23187-dependent perturbation of Ca(2+) homeostasis and the effector mechanisms of cell death...
Cytotoxicity of a mitochondriotropic quercetin derivative: mechanismsNicola Sassi
Department of Biomedical Sciences, University of Padova, Italy
Biochim Biophys Acta 1817:1095-106. 2012..Death induction was selective for fast-growing C-26 tumoral cells and mouse embryonic fibroblasts (MEFs) while sparing slow-growing MEFs. This suggests a possible use of Q-7BTPI as a chemotherapeutic agent...
The effects of idebenone on mitochondrial bioenergeticsValentina Giorgio
Department of Biomedical Sciences, University of Padova, Padova, Italy
Biochim Biophys Acta 1817:363-9. 2012..2011) PLoS One 6, e17963], electron transfer to complex III may allow reoxidation of NADH in complex I deficiencies...
Shedding light on the mitochondrial permeability transitionFernanda Ricchelli
Department of Biology, University of Padova, Padova, Italy
Biochim Biophys Acta 1807:482-90. 2011....
Early resistance to cell death and to onset of the mitochondrial permeability transition during hepatocarcinogenesis with 2-acetylaminofluorenePeter Christian Klohn
Department of Biomedical Sciences, University of Padua, Viale Giuseppe Colombo 3, I 35121 Padua, Italy
Proc Natl Acad Sci U S A 100:10014-9. 2003..Both events precede tumor formation, suggesting that suppression of apoptosis may contribute to the selection of a resistant phenotype, eventually increasing the probability of cell progression to the transformed state...
Assessing the molecular basis for rat-selective induction of the mitochondrial permeability transition by norbormideAlessandra Zulian
Department of Pharmacology and Anesthesiology Pharmacology Division, University of Padova, Padova, Italy
Biochim Biophys Acta 1767:980-8. 2007..The species-selectivity may depend on the unique properties of a transport system allowing drug internalisation in rat mitochondria...
Signal transduction to the permeability transition poreAndrea Rasola
Department of Biomedical Sciences and CNR Institute of Neuroscience, University of Padova, Italy
FEBS Lett 584:1989-96. 2010..Recent progress on PTP modulation by kinase/phosphatase signal transduction is discussed, with specific emphasis on hexokinase and on the Akt-ERK-GSK3 axis, which might modulate the PTP through CyP-D phosphorylation...
Serenoa repens extract targets mitochondria and activates the intrinsic apoptotic pathway in human prostate cancer cellsAntonella Baron
Venetian Institute of Molecular Medicine, University of Padova, Padova, Italy
BJU Int 103:1275-83. 2009....
Dimers of mitochondrial ATP synthase form the permeability transition poreValentina Giorgio
Consiglio Nazionale delle Ricerche Institute of Neuroscience and Department of Biomedical Sciences and Department of Biology, University of Padova, 35121 Padua, Italy
Proc Natl Acad Sci U S A 110:5887-92. 2013..Channel openings were inhibited by the ATP synthase inhibitor AMP-PNP (γ-imino ATP, a nonhydrolyzable ATP analog) and Mg(2+)/ADP. These results indicate that the PTP forms from dimers of the ATP synthase...
Mitochondrial energy dissipation by fatty acids. Mechanisms and implications for cell deathPaolo Bernardi
Department of Biomedical Sciences, Venetian Institute of Molecular Medicine, University of Padova, I-35131 Padova, Italy
Vitam Horm 65:97-126. 2002....
Effects of fatty acids on mitochondria: implications for cell deathDaniele Penzo
Department of Biomedical Sciences and CNR Institute for Neurosciences, University of Padova, Viale Giuseppe Colombo 3, I-35121 Padua, Italy
Biochim Biophys Acta 1555:160-5. 2002..With few exceptions, depolarization and cell death are due to opening of the PTP rather than to the direct effects of fatty acids on energy coupling...
The mitochondrial permeability transition pore and its involvement in cell death and in disease pathogenesisAndrea Rasola
CNR Institute of Neuroscience and Department of Biomedical Sciences, University of Padova, Viale Giuseppe Colombo 3, I 35121 Padua, Italy
Apoptosis 12:815-33. 2007..Here we review basic concepts about PTP structure, function and regulation within the framework of intracellular death signalling, and its role in disease pathogenesis...
Lentiviral-mediated RNAi in vivo silencing of Col6a1, a gene with complex tissue specific expression patternKosjenka Frka
Department of Histology Microbiology and Medical Biotechnology, University of Padova, Padova, Italy
J Biotechnol 141:8-17. 2009..Control of expression by the drug was stringent in many tissues; however, in some tissues turning off of siRNA synthesis was not complete. The data support the application of lentiviral vectors used here in transgenesis...
Mitochondria and reperfusion injury. The role of permeability transitionFabio Di Lisa
Istituto di Neuroscienze del CNR, Universita di Padova, Viale Giuseppe Colombo 3, 35121 Padua, Italy
Basic Res Cardiol 98:235-41. 2003....
Mitochondria and cardioprotectionFabio Di Lisa
Dipartimento di Chimica Biologica, Universita di Padova, Viale G Colombo 3, Padua 35121, Italy
Heart Fail Rev 12:249-60. 2007..Finally, mitochondrial targets for therapeutic intervention may include the switch of substrate being utilized, because inhibition of fatty acid oxidation is associated with cardioprotective effects...
Mitochondrial function and myocardial aging. A critical analysis of the role of permeability transitionFabio Di Lisa
Dipartimento di Chimica Biologica, Universita di Padova, Viale G Colombo 3, 35121 Padova, Italy
Cardiovasc Res 66:222-32. 2005....
Mitochondria and ischemia-reperfusion injury of the heart: fixing a holeFabio Di Lisa
Dipartimento di Chimica Biologica, Universita di Padova, Viale G Colombo 3, 35121 Padova
Cardiovasc Res 70:191-9. 2006..Here, we discuss our current understanding of the structure and function of PTP in the context of heart injury caused by ischemia and reperfusion...
Mitochondria as functional targets of proteins coded by human tumor virusesPaolo Bernardi
Department of Oncology and Surgical Sciences, University of Padova, Padova 35128, Italy
Adv Cancer Res 94:87-142. 2005....
Mitochondrial alterations induced by the p13II protein of human T-cell leukemia virus type 1. Critical role of arginine residuesLaura Ranzato
Department of Oncology and Surgical Sciences, University of Padova, Via Gattamelata 64, 35128 Padua, Italy
J Biol Chem 277:34424-33. 2002....
Species-specific modulation of the mitochondrial permeability transition by norbormideFernanda Ricchelli
C N R, Institute of Biomedical Technologies Padova Unit, Department of Biology, University of Padova, Viale Giuseppe Colombo 3, 35121 Padova, Italy
Biochim Biophys Acta 1708:178-86. 2005..The characterization of the norbormide target may lead to a better understanding of the mechanisms underlying the mitochondrial PTP as well as to the identification of species-specific drugs that affect mitochondrial function...
Induction of the permeability transition pore in cells depleted of mitochondrial DNAIonica Masgras
University of Padova, Padova, Italy
Biochim Biophys Acta 1817:1860-6. 2012..This article is part of a Special Issue entitled: 17th European Bioenergetics Conference (EBEC 2012)...
Bupivacaine myotoxicity is mediated by mitochondriaWilliam Irwin
Department of Biomedical Sciences, University of Padova and the Consiglio Nazionale delle Ricerche Units for the Study of Muscle Pathophysiology and Biomembranes, Viale Giuseppe Colombo 3, I-35121 Padova, Italy
J Biol Chem 277:12221-7. 2002....
The mitochondrial permeability transition porePaolo Bernardi
Department of Biomedical Sciences and CNR Institute of Neurosciences, University of Padova, Viale Giuseppe Colombo 3, I 35121 Padova, Italy
Novartis Found Symp 287:157-64; discussion 164-9. 2007..While the nature of the components forming the PTP remains controversial, the identification of novel inhibitors that can be used as affinity labels is offering new perspectives towards the molecular definition of the PTP...
BMAP-28, an antibiotic peptide of innate immunity, induces cell death through opening of the mitochondrial permeability transition poreAngela Risso
Department of Biotechnology and Biomedical Sciences, University of Udine, Via Cotonificio 108, I 33100 Udine, Italy
Mol Cell Biol 22:1926-35. 2002..Our results demonstrate that BMAP-28 is an inducer of the mitochondrial permeability transition pore and that its cytotoxic potential depends on its effects on mitochondrial permeability...
A p53-p66Shc signalling pathway controls intracellular redox status, levels of oxidation-damaged DNA and oxidative stress-induced apoptosisMirella Trinei
Department of Experimental Oncology, European Institute of Oncology, 20141 Milan, Italy
Oncogene 21:3872-8. 2002..We propose that steady-state levels of intracellular oxidants and oxidative damage are genetically determined and regulated by a stress-induced signal transduction pathway involving p53 and p66Shc...
Mitochondria are direct targets of the lipoxygenase inhibitor MK886. A strategy for cell killing by combined treatment with MK886 and cyclooxygenase inhibitorsArianna Gugliucci
Venetian Institute of Molecular Medicine, I-35129 Padova, Italy
J Biol Chem 277:31789-95. 2002..This approach should be specifically useful to kill cells possessing a high flux of arachidonic acid and its metabolites like prostate and colon cancer cells...
Ligand-selective modulation of the permeability transition pore by arginine modification. Opposing effects of p-hydroxyphenylglyoxal and phenylglyoxalMatts D Linder
Helsinki Biophysics and Biomembrane Group, Institute of Biomedicine, University of Helsinki, FIN-00014, Helsinki, Finland
J Biol Chem 277:937-42. 2002..The results indicate that both OH-PGO and PGO react with arginine at a stoichiometry of 2:1 and form stable adducts that may be feasible to identify the PTP at the molecular level...
Novel mitochondrial creatine transport activity. Implications for intracellular creatine compartments and bioenergeticsBernd Walzel
Swiss Federal Institute of Technology, , Institute of Cell Biology, , CH-8093 Zurich, Switzerland
J Biol Chem 277:37503-11. 2002..Our data suggest that mitochondria, shown here to possess creatine transport activity, may harbor such a creatine/phosphocreatine pool...
Cyclophilin D inactivation protects axons in experimental autoimmune encephalomyelitis, an animal model of multiple sclerosisMichael Forte
Vollum Institute, Oregon Health and Science University, Portland, OR 97239, USA
Proc Natl Acad Sci U S A 104:7558-63. 2007..Our results directly implicate pathological activation of the mitochondrial PTP in the axonal damage occurring during MS and identify CyPD, as well as the PTP, as a potential target for MS neuroprotective therapies...
The voltage-dependent anion channel is the target for a new class of inhibitors of the mitochondrial permeability transition poreAndrea M Cesura
Pharmaceutical Division, Drug Discovery Department, F Hoffmann La Roche Ltd, Grenzacherstrasse 124, CH 4070 Basel, Switzerland
J Biol Chem 278:49812-8. 2003..While Ro 68-3400 represents a new useful tool for the study of the structure and function of VDAC and the PTP, the results obtained provide direct evidence that VDAC1 is a component of this mitochondrial pore...
Yessotoxin, a shellfish biotoxin, is a potent inducer of the permeability transition in isolated mitochondria and intact cellsCristina Bianchi
Department of Biochemistry G. Moruzzi, University of Bologna, Via Irnerio 48, 40126 Bologna, Italy
Biochim Biophys Acta 1656:139-47. 2004....
Desensitization of the permeability transition pore by cyclosporin a prevents activation of the mitochondrial apoptotic pathway and liver damage by tumor necrosis factor-alphaMaria Eugenia Soriano
Consiglio Nazionale delle Ricerche Institute of Neuroscience at the Department of Biomedical Sciences, University of Padua, Viale Giuseppe Colombo 3, I-35121 Padua, Italy
J Biol Chem 279:36803-8. 2004....
Electron transfer between cytochrome c and p66Shc generates reactive oxygen species that trigger mitochondrial apoptosisMarco Giorgio
Experimental Oncology Department, European Institute of Oncology, Milan, Italy
Cell 122:221-33. 2005..These data demonstrate the existence of alternative redox reactions of the mitochondrial electron transfer chain, which evolved to generate proapoptotic ROS in response to specific stress signals...
Induction of the mitochondrial permeability transition by the DNA alkylating agent N-methyl-N'-nitro-N-nitrosoguanidine. Sorting cause and consequence of mitochondrial dysfunctionGiuliano Dodoni
Dipartimento di Chimica Biologica, , Viale G. Colombo 3, I-35121 Padova, Italy
Biochim Biophys Acta 1658:58-63. 2004..We conclude that a key event in MNNG-dependent cell death is induction of PTP opening that occurs independently of PARP-1 activation...
Modification of permeability transition pore arginine(s) by phenylglyoxal derivatives in isolated mitochondria and mammalian cells. Structure-function relationship of arginine ligandsMilena Johans
Helsinki Biophysics and Biomembrane Group, Institute of Biomedicine/Biochemistry, P.O. Box 63, University of Helsinki, Helsinki FIN-00014, Finland
J Biol Chem 280:12130-6. 2005..These results are consistent with a model where the target arginine plays a role as a voltage sensor...
Genetic dissection of the permeability transition poreMichael Forte
Vollum Institute, Oregon Health and Science University, 3181 SW Sam Jackson Park Road, Portland, OR 97239, USA
J Bioenerg Biomembr 37:121-8. 2005....
Cyclosporin A corrects mitochondrial dysfunction and muscle apoptosis in patients with collagen VI myopathiesLuciano Merlini
Department of Experimental and Diagnostic Medicine, Section of Medical Genetics, University of Ferrara, I 44100 Ferrara, Italy
Proc Natl Acad Sci U S A 105:5225-9. 2008....
Mitochondrial deoxynucleotide pool sizes in mouse liver and evidence for a transport mechanism for thymidine monophosphatePaola Ferraro
Departments of Biology and Biomedical Sciences, University of Padua, I-35131 Padua, Italy
Proc Natl Acad Sci U S A 103:18586-91. 2006..At nM external dTMP the nucleotide is concentrated 100-fold inside the mt matrix. Export of thymidine phosphates was much slower and possibly occurred at the level of dTDP...
Identification and characterization of a specific sensory epithelium in the rat larynxAndrea Sbarbati
Department of Morphological Biomedical Sciences, Human Anatomy and Histology Section, University of Verona, 37134 Verona, Italy
J Comp Neurol 475:188-201. 2004..The immunocytochemical and ultrastructural data suggested that SLSE is a chemoreceptor located in an optimal position for detecting substances entering the larynx from the pharynx or the trachea...
