Oxidative stress induces mitochondrial fragmentation in frataxin-deficient cells

Biochemical and Biophysical Research Communications, Available online 16 January 2012, In Press, Accepted Manuscript. http://dx.doi.org/10.1016/j.bbrc.2012.01.022

Sophie Lefevre a, b, 1, Dominika Sliwa a, 1, Pierre Rustin c, d, Jean-Michel Camadro a, Renata Santos a.
a Mitochondria, Metals and Oxidative Stress Laboratory, Institut Jacques Monod, CNRS-Université Paris-Diderot, Sorbonne Paris Cité, 15 rue Hélène Brion, 75205 Paris cedex 13, France
b ED515 UPMC, 4 place Jussieu 75005 Paris, France
c Inserm, U676, Physiopathology and Therapy of Mitochondrial Disease Laboratory, 75019 Paris, France
d Université Paris-Diderot, Faculté de Médecine Denis Diderot, IFR02, Paris, France

Keywords: Friedreich ataxia (FA), mitochondrial frataxin, iron-sulfur cluster assembly, fragmented mitochondria, oxidative stress, mitochondrial dynamics.

Hippotherapy: An Intervention to Habilitate Balance Deficits in Children With Movement Disorders--A Clinical Trial.

Phys Ther. 2012 Jan 12, Published online before print, doi: 10.2522/​ptj.20110081

Silkwood-Sherer DJ, Killian CB, Long TM, Martin KS.

D.J Silkwood-Sherer, PT, DHS, HPCS, Graduate Program in Physical Therapy, Herbert H. and Grace A. Dow College of Health Professions, 1202 Health Professions Bldg, Central Michigan University, Mt Pleasant, MI 48859 (USA).

Keywords: hippotherapy, children with movement disorders, postural instability, mild to moderate balance problems, Activities Scale for Kids-Performance (ASKp), daily life skills.


Hippotherapy may be a viable treatment strategy for children with mild to moderate balance problems to improve balance deficits and increase performance of daily life skills.

Effects of Friedreich's ataxia GAA repeats on DNA replication in mammalian cells

Nucl. Acids Res. (2012) doi: 10.1093/nar/gks021.

Gurangad S. Chandok 1, Mayank P. Patel 1, Sergei M. Mirkin 2 and Maria M. Krasilnikova
Department of Biochemistry and Molecular Biology, Penn State University, University Park, PA 16802 and 2Department of Biology, Tufts University.

Keyword: Friedreich's ataxia (FRDA),(GAA)n repeat, age-dependent instability, episomal DNA replication, chromatin.

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Reactive Oxygen Species and Aging in Caenorhabditis elegans: Causal or Casual Relationship?

Jeremy Michael Van Raamsdonk and Siegfried Hekimi and. Antioxidants & Redox Signaling. December 15, 2010, 13(12): 1911-1953. doi:10.1089/ars.2010.3215.

Keywords: reactive oxygen species (ROS), aging, Caenorhabditis elegans, frh-1-frataxin

S-Glutathionylation: From Molecular Mechanisms to Health Outcomes

Antioxidants & Redox Signaling. July 1, 2011, 15(1): 233-270. doi:10.1089/ars.2010.3540.

Ying Xiong, Joachim D. Uys, Kenneth D. Tew, and Danyelle M. Townsend.

KEYWORDS: Redox homeostasis, human disease pathologies, glutathione, Friedreich's ataxia

Mechanisms of Altered Redox Regulation in Neurodegenerative Diseases—Focus on S-Glutathionylation

Antioxidants & Redox Signaling. null, Vol. 0, No. 0,-Not available-, ahead of print. doi:10.1089/ars.2011.4119.

Elizabeth A. Sabens Liedhegner 1, Xing-Huang Gao 2, and John J. Mieyal 2,3
1Neuroscience Research Center, Medical College of Wisconsin, Milwaukee, Wisconsin.
2Department of Pharmacology, Case Western Reserve University, School of Medicine, Cleveland, Ohio.
3Louis B. Stokes Veterans Affairs Medical Research Center, Cleveland, Ohio.



Keywords: Neurodegenerative diseases, oxidative stress, reactive oxygen species (ROS), reactive nitrogen species (RNS), glutathione (GSH), Friedreich's ataxia

The European Hospital Exemption Clause—New Option for Gene Therapy?

Human Gene Therapy.
Christian J. Buchholz, Ralf Sanzenbacher, and Silke Schüle. January 2012, 23(1): 7-12. doi:10.1089/hum.2011.2529.

Keywords: Gene-therapy medicinal products, authorized clinical trials, centralized procedure, European Medicines Agency, Regulation (EC) No. 1394/2007, cell-therapy products, hospital exemption, exclusive professional responsibility.

Friedreich-Like Ataxia as an Initial Manifestation of Mitochondrial DNA 8344A>G Mutation.

J Child Neurol. 2012 Jan 12.

Chevallier JA, Koenig MK.
Department of Pediatrics, Division of Child and Adolescent Neurology, University of Texas Health Science Center, Houston, TX, USA.

Keywords: subacute onset of ataxia, acute-onset cardiac and pulmonary failure, Magnetic resonance imaging (MRI),nerve conduction velocities, electromyography, sensorimotor axonal neuropathy, Friedreich ataxia, molecular testing of the frataxin gene, mutation at 8344A>G in transfer RNA lysine.

Iron Intake In Teen Years Can Impact Brain In Later Life

University of California - Los Angeles. (2012, January 13). "Iron Intake In Teen Years Can Impact Brain In Later Life." Medical News Today

"The findings may aid future studies of how iron transport affects brain function, development and the risk of neurodegeneration."

Reference:
Brain structure in healthy adults is related to serum transferrin and the H63D polymorphism in the HFE gene. Published online before print January 9, 2012, doi: 10.1073/pnas.1105543109 PNAS January 9, 2012

DEVELOPPEMENT D’UN SCORE DE STABILITE CHEZ LES PERSONNES PRESENTANT DES PATHOLOGIES D’ORIGINE NEUROLOGIQUE ENTRAINANT DES TROUBLES DE LA MARCHE ET/OU DE L’EQUILIBRE

THESE, Pour l’obtention du grade de DOCTEUR DE L’UNIVERSITE PARIS-SUD 11
Arnaud GOUELLE

Soutenue le 13 décembre 2011


CHAPITRE VI - Apport du GVI dans l’évaluation de l’ataxie de Friedreich 155
VI.1 Préambule 155
VI.2 Introduction 157
VI.3 Méthode 158
VI.4 Résultats 160
VI.5 Discussion 163