Fifth Congress of the International BioIron Society (IBIS) (BioIron 2013) at University College London.

Fifth Congress of the International BioIron Society (IBIS) (BioIron 2013) at
University College London. 2013 Program Book - International BioIron Society, April 14-18

Podium #36
IRP-1 CONTROLS IRON METABOLISM IN FRATAXIN-DEFICIENT TISSUES
Alain Martelli, PhD, Stéphane Schmucker, PhD, Laurence Reutenauer, Hervé Puy, Prof., Bruno Galy, PhD, Matthias
Hentze, Prof. and Hélène Puccio, Research Director

Podium #47
ABNORMAL BODY IRON DISTRIBUTION AND ERYTHROPOIESIS IN A NOVEL MOUSE MODEL WITH INDUCIBLE GAIN
OF IRON REGULATORY PROTEIN (IRP) -1 FUNCTION
Daniela Casarrubea, MSc, Lydie Viatte, PhD, Tina Hallas, Aparna Vasanthakumar, PhD, Richard S. Eisenstein, PhD, Klaus
Schümann, PhD, Matthias W. Hentze, PhD and Bruno Galy, PhD

Podium #66
IDENTIFICATION OF NONFERRITIN MITOCHONDRIAL IRON DEPOSITS IN A MOUSE MODEL OF FRIEDREICH ATAXIA
Des R. Richardson, M. Whitnall, Y. Suryo Rahmant, M.L.H. Huang, F. Saletta, H.C. Lok, L. Gutierrez, F.J. Lazaro, A.J.
Fleming, T.G. St Pierre, M.R. Mikhael and P. Ponka

Poster #34
MITOCHONDRIAL PROTEIN, FRATAXIN, IS DOWNREGULATED IN HEMODIALYSIS PATIENTS
Takeshi Nakanishi, MD, PhD; Yukiko Hasuike, MD,PhD; Soshi Yorifuji, MD; Ayako Matsumoto, MD; Mana Yahiro, MD, PhD;
Aritoshi Kida, MD; Yasuyuki Nagasawa, MD, PhD; Takahiro Kuragano, MD, PhD

Poster #35
FRATAXIN IS A REGULATOR OF THE FE-S BIOGENESIS
Salvatore Adinolfi, biologist

Poster #39
MITOCHONDRIA-TARGETED IRON CHELATORS FOR THE MONITORING AND ADJUSTMENT OF CELLULAR LABILE
IRON POOLS
Vincenzo Abbate, PhD; Robert Hider, PhD; Charareh Pourzand, PhD; Olivier Reelfs, PhD

Poster #62
DOWN REGULATION OF RESPIRATORY ENZYMES IN FRATAXIN DEFICIENT YEAST IS MEDIATED BY THE
METABOLIC REGULATORS ADR1 AND CTH2
David Alsina, Armando Moreno-Cermeño, Elia Obis, Joaquim Ros and Jordi Tamarit

Poster #64
DEFINING THE ARCHITECTURE OF THE MITOCHODRIAL IRON-SULFUR CLUSTER ASSEMBLY MACHINERY
Belinda Galean

Poster #214
A CELL MODEL FOR FRIEDREICH ATAXIA USING DORSAL ROOT GANGLIA NEURONS
Stefka Mincheva-Tasheva, PhD, Elia Obis, Joaquim Ros, PhD and Jordi Tamarit, PhD

Poster #231
NOVEL LIGHT-ACTIVATED CAGED IRON CHELATORS: TARGETED PRODRUGS FOR IRON RELATED-DISORDERS
Benjamin Young, MPharm, Olivier Reelfs, PhD, DSc, Asma Aroun, PhD, Seyed Ali Miri, BSc, Magnus Hoffmann, BSc,
Charareh Pourzand, PhD, DSc and Ian Eggleston

Poster #273
A PRIMARY CULTURE OF CARDIOMYOCYTES WITH FRATAXIN DEFICIENCY EXHIBITS A METABOLIC SWITCH
BEFORE IRON DISARRANGEMENTS
Elia Obis, David Alsina, Joaquim Ros and Jordi Tamarit

Therapeutic Strategies in Friedreich's Ataxia

Therapeutic Strategies in Friedreich's Ataxia. Timothy E. Richardson, Heather N. Kelly, Amanda E. Yu, James W. Simpkins; Brain Research, Available online 13 April 2013. http://dx.doi.org/10.1016/j.brainres.2013.04.005

Keywords: Friedreich's ataxia (FA), FXN, frataxin, therapeutic research, ongoing treatment strategies, 17β-estradiol, methylene blue.

Cardiomyopathy and the electrocardiogram in Friedreich's ataxia

Cardiomyopathy and the electrocardiogram in Friedreich's ataxia . Alexandra H Wood, Simon W Dubrey; British Journal of Hospital Medicine, Vol. 74, Iss. 4, 09 Apr 2013, pp 232 - 233

Iron uptake in quiescent and inflammation-activated astrocytes: A potentially neuroprotective control of iron burden

Iron uptake in quiescent and inflammation-activated astrocytes: A potentially neuroprotective control of iron burden. Ilaria Pelizzoni, Daniele Zacchetti, Alessandro Campanella, Fabio Grohovaz, Franca Codazzi; Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, Available online 11 April 2013. http://dx.doi.org/10.1016/j.bbadis.2013.04.007

OPEN ACCESS

Keywords: Astrocytes, NTBI, iron uptake, DMT1, activation process, TRP channels, neuroinflammation

Vitamin Switches on a Gene, A Potential New Treatment for Friedreich's Ataxia?

Vitamin Switches on a Gene, A Potential New Treatment for Friedreich's Ataxia?. MRC Clinical Sciences Centre, Faculty of Medicine, Imperial College London.

Novel research from the CSC holds promise for a new therapeutic approach, using a commonly available vitamin supplement to modify the epigenetic controls of the genetic defect causing the illness.

Related to: Heterochromatinization induced by GAA-repeat hyperexpansion in Friedreich's ataxia can be reduced upon HDAC inhibition by Vitamin B3

Frataxin Deficiency Leads to Reduced Expression and Impaired Translocation of NF-E2-Related Factor (Nrf2) in Cultured Motor Neurons

Frataxin Deficiency Leads to Reduced Expression and Impaired Translocation of NF-E2-Related Factor (Nrf2) in Cultured Motor Neurons. D'Oria, V.; Petrini, S.; Travaglini, L.; Priori, C.; Piermarini, E.; Petrillo, S.; Carletti, B.; Bertini, E.; Piemonte, F.; International Journal of Molecular Sciences. 2013; 14(4):7853-7865.

OPEN ACCESS

Mitochondria targeted therapeutic approaches in Parkinson's and Huntington's diseases

Mitochondria targeted therapeutic approaches in Parkinson's and Huntington's diseases. Rajnish K. Chaturvedi, M. Flint Beal. Molecular and Cellular Neuroscience, Volume 55, July 2013, Pages 101-114. http://dx.doi.org/10.1016/j.mcn.2012.11.011

Keywords: Parkinson's disease, Huntington's disease, Neurodegenerative diseases, Mitochondrial dysfunction, Creatine, Co-Q10, PGC-1α, Sirtuins.

Cerebello-cerebral connectivity deficits in Friedreich ataxia

Cerebello-cerebral connectivity deficits in Friedreich ataxia. Andrew Zalesky, Hamed Akhlaghi, Louise A. Corben, John L. Bradshaw, Martin B. Delatycki, Elsdon Storey, Nellie Georgiou-Karistianis, Gary F. Egan. Brain Structure and Function, April 2013. DOI 10.1007/s00429-013-0547-1

Keywords: Friedreich ataxia, Diffusion-weighted imaging, White matter, Connectome, Connectivity, supplementary motor area, cingulate cortex, frontal cortices, putamen, other subcortical nuclei.

Mitochondrial Diseases of the Brain

Mitochondrial Diseases of the Brain. Rajnish K. Chaturvedi, M. Flint Beal; Free Radical Biology and Medicine, Available online 6 April 2013.
http://dx.doi.org/10.1016/j.freeradbiomed.2013.03.018

Keywords: Parkinson’s disease, Alzheimer’s disease, Huntington’s disease, Amyotrophic lateral sclerosis, Charcot-Marie-Tooth disease, Friedreich’s ataxia, Neurodegenerative diseases, Mitochondrial dysfunction, Creatine, Co-Q10, PGC-1α, Sirtuins.

Moving Forward on Shifting Sands: Ethical Regulation of Gene Therapy Clinical Trials in the United Kingdom

Moving Forward on Shifting Sands: Ethical Regulation of Gene Therapy Clinical Trials in the United Kingdom. Emma Morris, Martin Gore, Andrew Baker and Adrian J Thrasher; Molecular Therapy (2013); 21 4, 715–716. doi:10.1038/mt.2013.43

Editorial. FULL TEXT