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Mitochondrial Energetics and Therapeutics

Douglas C. Wallace, Weiwei Fan, Vincent Procaccio
2010 Annual Review of Pathology  
Mitochondrial dysfunction has been linked to a wide range of degenerative and metabolic diseases, cancer, and aging. All these clinical manifestations arise from the central role of bioenergetics in cell biology. Although genetic therapies are maturing as the rules of bioenergetic genetics are clarified, metabolic therapies have been ineffectual. This failure results from our limited appreciation of the role of bioenergetics as the interface between the environment and the cell. A systems
more » ... ch, which, ironically, was first successfully applied over 80 years ago with the introduction of the ketogenic diet, is required. Analysis of the many ways that a shift from carbohydrate glycolytic metabolism to fatty acid and ketone oxidative metabolism may modulate metabolism, signal transduction pathways, and the epigenome gives us an appreciation of the ketogenic diet and the potential for bioenergetic therapeutics. 297 Annu. Rev. Pathol. Mech. Dis. 2010.5:297-348. Downloaded from www.annualreviews.org by Florida State University on 07/18/11. For personal use only.
doi:10.1146/annurev.pathol.4.110807.092314 pmid:20078222 pmcid:PMC3245719 fatcat:g7ixcue4jjfa3gumdrthn7ssjy

Dominant optic atrophy

Guy Lenaers, Christian P Hamel, Cecile Delettre, Patrizia Amati-Bonneau, Vincent Procaccio, Dominique Bonneau, Pascal Reynier, Dan Milea
2012 Orphanet Journal of Rare Diseases  
doi:10.1186/1750-1172-7-46 pmid:22776096 pmcid:PMC3526509 fatcat:anuechjmcrfyjoin6caxp64dvy

Multiplex analysis of mitochondrial DNA pathogenic and polymorphic sequence variants

Jason C. Poole, Vincent Procaccio, Martin C. Brandon, Greg Merrick, Douglas C. Wallace
2010 Biological chemistry  
doi:10.1515/bc.2010.125 pmid:20707610 pmcid:PMC4023691 fatcat:htxblgqqpjb4llphgr3boec6ce

Estrogen suppresses brain mitochondrial oxidative stress in female and male rats

Ali Razmara, Sue P. Duckles, Diana N. Krause, Vincent Procaccio
2007 Brain Research  
Procaccio, unpublished observations).  ...  Procaccio, unpublished observations).  ... 
doi:10.1016/j.brainres.2007.08.036 pmid:17889838 pmcid:PMC2099309 fatcat:6mbbct6nhnfefer3ab5fugevgq

How Can a Ketogenic Diet Improve Motor Function?

Charlotte Veyrat-Durebex, Pascal Reynier, Vincent Procaccio, Rudolf Hergesheimer, Philippe Corcia, Christian R. Andres, Hélène Blasco
2018 Frontiers in Molecular Neuroscience  
A ketogenic diet (KD) is a normocaloric diet composed by high fat (80-90%), low carbohydrate, and low protein consumption that induces fasting-like effects. KD increases ketone body (KBs) production and its concentration in the blood, providing the brain an alternative energy supply that enhances oxidative mitochondrial metabolism. In addition to its profound impact on neuro-metabolism and bioenergetics, the neuroprotective effect of specific polyunsaturated fatty acids and KBs involves
more » ... pic mechanisms, such as the modulation of neuronal membrane excitability, inflammation, or reactive oxygen species production. KD is a therapy that has been used for almost a century to treat medically intractable epilepsy and has been increasingly explored in a number of neurological diseases. Motor function has also been shown to be improved by KD and/or medium-chain triglyceride diets in rodent models of Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, and spinal cord injury. These studies have proposed that KD may induce a modification in synaptic morphology and function, involving ionic channels, glutamatergic transmission, or synaptic vesicular cycling machinery. However, little is understood about the molecular mechanisms underlying the impact of KD on motor function and the perspectives of its use to acquire the neuromuscular effects. The aim of this review is to explore the conditions through which KD might improve motor function. First, we will describe the main consequences of KD exposure in tissues involved in motor function. Second, we will report and discuss the relevance of KD in pre-clinical and clinical trials in the major diseases presenting motor dysfunction.
doi:10.3389/fnmol.2018.00015 pmid:29434537 pmcid:PMC5790787 fatcat:ft5xfnca5ngizm5yhm5twpyqqq

Yeast models of ATPase-based diseases

Roza Kucharczyk, Nahia Ezkurdia, Elodie Couplan, Vincent Procaccio, Sharon H. Ackerman, Marc Blondel, Jean-Paul di Rago
2010 Biochimica et Biophysica Acta - Bioenergetics  
Procaccio 6,7 , Sharon H.  ...  human cells deficient in ATP synthase have been found [1] . doi:10.1016/j.bbabio.2010.04.312 12L.2 Yeast models of ATPase-based diseases Roza Kucharczyk 1 , Nahia Ezkurdia 1 , Elodie Couplan 2,3,4,5 , Vincent  ... 
doi:10.1016/j.bbabio.2010.04.313 fatcat:snx2jqofxjcotmr4qavgtysn6i

Is ABCC6 a genuine mitochondrial protein?

Marc Ferré, Pascal Reynier, Arnaud Chevrollier, Delphine Prunier-Mirebeau, Georges Lefthériotis, Daniel Henrion, Dominique Bonneau, Vincent Procaccio, Ludovic Martin
2013 BMC Research Notes  
, Vincent [8], Martin, Ludovic [9] Type Article scientifique dans une revue à comité de lecture Année 2013 Langue Anglais Date 2013 Volume 6 Titre de la revue BMC research notes ISSN  ...  publication Article de revue Auteur Ferré, Marc [1], Reynier, Pascal [2], Chevrollier, Arnaud [3], Mirebeau-Prunier, Delphine [4], Lefthériotis, Georges [5], Henrion, Daniel [6], Bonneau, Dominique [7], Procaccio  ... 
doi:10.1186/1756-0500-6-427 pmid:24152371 pmcid:PMC4015885 fatcat:rbektu5crzberaaz7eym4n64zq

De la levure aux maladies neurodégénératives

Guy Lenaers, Patrizia Amati-Bonneau, Cécile Delettre, Arnaud Chevrollier, Christophe Verny, Dan Miléa, Vincent Procaccio, Dominique Bonneau, Christian Hamel, Pascal Reynier
2010 M S.Médecine Sciences  
doi:10.1051/medsci/20102610836 pmid:20929674 fatcat:qewihr5syfcmrlalp333ueaylu

Sensorineural hearing loss in OPA1-linked disorders

Stéphanie Leruez, Dan Milea, Sabine Defoort-Dhellemmes, Estelle Colin, Martine Crochet, Vincent Procaccio, Marc Ferré, Julie Lamblin, Valérie Drouin, Catherine Vincent-Delorme, Guy Lenaers, Christian Hamel (+7 others)
2013 Brain  
doi:10.1093/brain/aws340 pmid:23384603 fatcat:wsxxwojzwzcnxe6trdpxrgka4m

Increased prevalence of val66met BDNF genotype among subjects with cervical dystonia

Steven C. Cramer, Ajay Sampat, Maureen Haske-Palomino, Shawn Nguyen, Vincent Procaccio, Neal Hermanowicz
2010 Neuroscience Letters  
doi:10.1016/j.neulet.2009.10.059 pmid:19857550 pmcid:PMC3270368 fatcat:dy5ei6cxtvdptp7hapryw3egj4

Role of SUV3 Helicase in Maintaining Mitochondrial Homeostasis in Human Cells

Lily Khidr, Guikai Wu, Antonio Davila, Vincent Procaccio, Douglas Wallace, Wen-Hwa Lee
2008 Journal of Biological Chemistry  
Procaccio, D. Wallace, and W.-H. Lee, unpublished data.  ... 
doi:10.1074/jbc.m802991200 pmid:18678873 pmcid:PMC2556002 fatcat:pqnuetkizbgzbfasggybyhubqa

IL-26 Confers Proinflammatory Properties to Extracellular DNA

Caroline Poli, Jean François Augusto, Jonathan Dauvé, Clément Adam, Laurence Preisser, Vincent Larochette, Pascale Pignon, Ariel Savina, Simon Blanchard, Jean François Subra, Alain Chevailler, Vincent Procaccio (+6 others)
2017 Journal of Immunology  
doi:10.4049/jimmunol.1600594 pmid:28356384 fatcat:ei5z34x6unfgxdvxytd7bbzhra

Predicting the pathogenicity of novel variants in mitochondrial tRNA with MitoTIP

Sanjay Sonney, Jeremy Leipzig, Marie T. Lott, Shiping Zhang, Vincent Procaccio, Douglas C. Wallace, Neal Sondheimer, Timothée Poisot
2017 PLoS Computational Biology  
Lott, Vincent Procaccio. Formal analysis: Sanjay Sonney. Table 1 . 1 Comparison between predictive systems.  ... 
doi:10.1371/journal.pcbi.1005867 pmid:29227991 pmcid:PMC5739504 fatcat:476a5c7ocvgm7kdmymxn27qau4

Succinate Dehydrogenase Is a Direct Target of Sirtuin 3 Deacetylase Activity

Lydia W. S. Finley, Wilhelm Haas, Valérie Desquiret-Dumas, Douglas C. Wallace, Vincent Procaccio, Steven P. Gygi, Marcia C. Haigis, Paul Cobine
2011 PLoS ONE  
doi:10.1371/journal.pone.0023295 pmid:21858060 pmcid:PMC3157345 fatcat:jasagj2zz5gz3jgm4tlmv4fqla

BDNF val66met polymorphism is associated with modified experience-dependent plasticity in human motor cortex

Jeffrey A Kleim, Sheila Chan, Erin Pringle, Kellan Schallert, Vincent Procaccio, Richard Jimenez, Steven C Cramer
2006 Nature Neuroscience  
doi:10.1038/nn1699 pmid:16680163 fatcat:t46gvzsntbeilf5n2pkknew6ty
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