Human Ind1, an iron-sulfur cluster assembly factor for respiratory complex I.

Mol Cell Biol. 2009 Sep 14.

Sheftel AD, Stehling O, Pierik AJ, Netz DJ, Kerscher S, Elsässer HP, Wittig I, Balk J, Brandt U, Lill R.

Institut für Zytobiologie, Philipps-Universität Marburg, Robert-Koch-Strasse 6, 35033 Marburg, Germany; Goethe-Universität, Zentrum der Biologischen Chemie, Molekulare Bioenergetik, Cluster of Excellence "Macromolecular Complexes", 60590 Frankfurt am Main, Germany; Department of Plant Sciences, University of Cambridge, Downing Street, Cambridge CB2 3EA, UK.

Keywords: Respiratory complex I, NADH:ubiquinone oxidoreductase, iron-sulfur (Fe/S) clusters, mitochondrial diseases,  Fe/S cofactors, huInd1, HeLa cells,  RNAi technology, NDUFS1, NDUFV1, NDUFS3, NDUFA13, radiolabelling technique, iron.

El factor neurotrófico BDNF disminuye la neurodegeneración inducida por la deficiencia de frataxina en cultivos neuronales.

(The neurotrophic factor BDNF reduces neurodegeneration induced by frataxin deficiency in neuronal cultures. )



Comunicacion: (sección Bases Moleculares de la Patología )

Y.M. Katsu, F. Lim, J. Díaz-Nido
UAM-CSIC, Madrid.

 
XXXII Congreso de la Sociedad Española de Bioquímica y Biología Molecular (SEBBM)
Oviedo, 23-26 de septiembre, 2009

Friedreich's ataxia: Oxidative stress and cytoskeletal abnormalities

Journal of the Neurological Sciences
Article in Press, Corrected Proof

  Marco Sparaco^{a, 1}, Laura Maria Gaeta^{b, 1}, Filippo Maria Santorelli^b, Chiara Passarelli^b, Giulia Tozzi^b, Enrico Bertini^b, Alessandro Simonati^c, Francesco Scaravilli^d, Franco Taroni^e, Charles Duyckaerts^f, Michele Feleppa^a and Fiorella Piemonte^{b, ,}

^aDivision of Neurology, Department of Neurosciences, Azienda Ospedaliera “G. Rummo”, 82100 Benevento, Italy
^bMolecular Medicine Unit, Children's Hospital and Research Institute “Bambino Gesù”, Roma, Italy
^cDepartment of Neurological and Visual Sciences, Section of Neurology, University of Verona, Policlinico G.B. Rossi, 37134 Verona, Italy
^dDivision of Neuropathology, Institute of Neurology, University College London, WC1N 3BG London, UK
^eUO Biochimica e Genetica, Fondazione IRCCS Istituto Neurologico “Carlo Besta”, Milano, Italy
^fLaboratoire de Neuropathologie Raymond Escourolle, Hôpital de La Salpêtrière, 75651 Paris, France

Received 2 April 2009; 

revised 24 July 2009; 

accepted 13 August 2009. 

Available online 12 September 2009.

Keywords: Glutathione; Oxidative stress; Friedreich's ataxia; Cytoskeletal proteins,  frataxin, Complexes I, II, III,  aconitase, fibroblasts, tubulin,  neurofilaments, motor neurons.

The Iron−Sulfur Cluster of Pyruvate Formate-Lyase Activating Enzyme in Whole Cells: Cluster Interconversion and a Valence-Localized [4Fe-4S]2+ State

Biochemistry, Article ASAP

DOI: 10.1021/bi9010286

Publication Date (Web): August 27, 2009

Jian Yang‡, Sunil G. Naik§, Danilo O. Ortillo§, Ricardo Garca-Serres§, Meng Li‡, William E. Broderick, Boi Hanh Huynh*§ and Joan B. Broderick*

Department of Chemistry and Biochemistry, Montana State University, Bozeman, Montana 59718

^‡ Department of Chemistry, Michigan State University, East Lansing, Michigan 48824

^§ Department of Physics, Emory University, Atlanta, Georgia 30322

KEYWORDS: Pyruvate formate-lyase activating enzyme (PFL-AE),  glycyl radical, pyruvate formate-lyase (PFL),  [4Fe-4S] cluster, cluster interconversions,  [4Fe-4S]²⁺  , [2Fe-2S]²⁺ , Fe^III,  Fe^II,  redox state, 5′-deoxyadenosine, AMP, ADP, and methylthioadenosine, oxidative damage.

Recurrent Ventricular Tachycardia in Patient with Friedreich's Ataxia in the Absence of Clinical Myocardial Disease.

Pacing Clin Electrophysiol. 2009 Sep 10

Asaad N, El-Menyar A, Al Suwaidi J.

From the Department of Cardiology and Cardiovascular Surgery, Hamad General Hospital and Weill Cornell Medical College, Doha, Qatar.

Uptake of ferrous iron by cultured rat astrocytes

Journal of Neuroscience Research, Published Online: 10;Sep;2009


Ketki Tulpule ^{1 2}, Stephen R. Robinson ³, Glenda M. Bishop ³, Ralf Dringen ^{1 2 3 *1}Center for Biomolecular Interactions Bremen, University of Bremen, Bremen, Germany
²Center for Environmental Research and Sustainable Technology, Bremen, Germany
³School of Psychology, Psychiatry and Psychological Medicine, Monash University, Clayton, Victoria, Australia

TARGETING NUCLEIC ACIDS IN BACTERIA WITH SYNTHETIC LIGANDS

Abbas Nikravesh (Thesis for doctoral degree (Ph.D.))         
Stockholm 2008
From the Programme for Genomics and Bioinformatics
Department of Cell and Molecular Biology
Karolinska Institutet, Stockholm, Sweden



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3.4 PAPER IV
Friedreich’s ataxia (GAA)n triplex DNA structures are stabilized by Benzoquinoquinoxaline derivatives

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5 FUTURE PERSPECTIVES
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The experiments on (GAA)n repeat sequences described in this thesis were restricted to in vitro studies and probing of E. coli plasmid DNA. Nevertheless, the evidence that we provide for the existence of triplex structures within FA (GAA)n repeats suggests that destabilization of these structures could provide a possible therapeutic strategy for patients. Whereas BQQ stabilizes triplex structures, other DNA ligands may destabilize triplex structures. Such strategies may offer hope for developing small molecule DNA ligands to treat FA or other conditions that involve triplex DNA structures that arise from tri nucleotide repeat expansions.

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Structure-Specific Recognition of Friedreich's Ataxia (GAA)n Repeats by Benzoquinoquinoxaline Derivatives

ChemBioChem, Published Online: 10 Sep 2009

Helen Bergquist 1, Abbas Nikravesh, Dr. 2, Raquel Domingo Fernández 1, Veronica Larsson 1, Chi-Hung Nguyen, Dr. 3, Liam Good, Dr. 2 4, Rula Zain, Dr. 1 *


1Department of Molecular Biology and Functional Genomics, Stockholm University, Svante Arrhenius väg 20C, 10691 Stockholm (Sweden), Fax: (+46) 8-166488

2Department of Cell and Molecular Biology, Karolinska Institutet, Berzelius väg 35, 17177 Stockholm (Sweden)

3UMR176 CNRS-Institut Curie, Laboratoire de Pharmacochimie, Centre Universitaire, Bâtiment 110, 91405 Orsay (France)

4Department of Pathology and Infectious Diseases, Royal Veterinary College, University of London, Hawkshead Lane, N. Mymms AL9 7TA (UK)

Friedreich’s Ataxia Symposium 2009 - Children’s Hospital of Philadelphia

The symposium will take place on November 13 and 14.
Further information can be obtained from the Continuing Medical Education Department at the Children’s Hospital of Philadelphia at (215) 590 5263.

Elucidation of the mechanism of mitochondrial iron loading in Friedreich's ataxia by analysis of a mouse mutant

Proceedings of the National Academy of Science USA

Edited by Solomon H. Snyder, Johns Hopkins University School of Medicine, Baltimore, MD, and approved July 30, 2009 (received for review June 17, 2009)

Michael Li-Hsuan Huanga, Erika M. Beckera, Megan Whitnalla, Yohan Suryo Rahmantoa, Prem Ponkab,1 and Des R. Richardson.

Keywords: muscle creatine kinase (MCK), frataxin knockout mouse, frataxin deficiency, iron metabolism, neurologic and cardiologic degeneration, iron-sulfur cluster (ISC) synthesis, cysteine desulferase Nfs1, mitochondrial ferritin, 5-aminolevulinate dehydratase, coproporphyrinogen oxidase, hydroxymethylbilane synthase, uroporphyrinogen III synthase, ferrochelatase, Sec15l1, mitoferrin-2 (Mfrn2).