Thursday, November 3, 2011

Specific Alterations of Carbohydrate Metabolism Are Associated With Hepatocarcinogenesis in Mitochondrially Impaired Mice

Hum. Mol. Genet. (2011) doi: 10.1093/hmg/ddr499

René Thierbach, Simone Florian, Katharina Wolfrum, Anja Voigt, Gunnar Drewes, Urte Blume, Peter Bannasch, Michael Ristow, Pablo Steinberg

Keywords: Friedreich's ataxia, AlbFxn-/- mice, ATP, carbohydrate metabolism, Frataxin, gene disruption, glucose-6-phosphate, glucose transport, glycogen, glycolysis, mitochondria.

Wednesday, November 2, 2011

Friedreich Ataxia - Pipeline Review, H2 2011

Published by Global Markets Direct on Oct 26, 2011 , 54 pages

This report provides an overview on the therapeutic development for Friedreich Ataxia, it show the Friedreich Ataxia therapeutic pipeline, with latest updates, and late-stage and discontinued projects.

Tuesday, November 1, 2011

The Role of Mitochondrial Dysfunction in the Pathogenesis of Friedreich's Ataxia

Egypt J. Neurol. Psychiat. Neurosurg. July 2011 Vol. 48 3 : 229 - 234

Hasan G. Nassar,Wael A. Fadel,Wafaa Ibrahim,Wafik S Bahnasy

Keywords: Friedreich's ataxia, Glutathione, Mitochondrial Complexes.

Full text pdf

Sunday, October 30, 2011

Evaluation of Myocardial Motion in Friedreich Ataxia Patients and Healthy Volunteers by High Temporal and Spatial Resolution MRI Tissue Phase-mapping Imaging

RSNA Meeting, November 25-30, McCormick Place, Chicago, Illinois
Scientific Informal (Poster) Presentations

Author List: Karsten Kortuem | Christian Oliver Ritter MD | Frank Weidemann | Herbert Koestler PhD | Dietbert Hahn MD | Meinrad Johannes Beer MD

Friday, October 28, 2011

Oligomerization propensity and flexibility of yeast frataxin studied by X-ray crystallography and small-angle X-ray scattering

Journal of Molecular Biology, In Press, Accepted Manuscript, doi:10.1016/j.jmb.2011.10.034

Christopher A.G. Söderberg a, [1], Alexander V. Shkumatov b, 2, Sreekanth Rajan a, Oleksandr Gakh c, Dmitri I. Svergun b, Grazia Isaya c,, Salam Al-Karadaghi a.
a Center for Molecular Protein Science, Institute for Chemistry and Chemical Engineering, Lund University, PO Box 124, SE-221 00 Lund, Sweden
b EMBL, Hamburg Outstation, Notkestraße 85, D-22603 Hamburg, Germany
c Department of Pediatric and Adolescent Medicine and Department of Biochemistry and Molecular Biology, Mayo Clinic, Rochester, MN 55905, USA

Keywords: Protein oligomerization; Protein flexibility; metal chaperone; Friedreich's ataxia; Neurodegenerative diseases

The cerebellar cognitive profile

Brain (2011) first published online October 27, 2011 doi:10.1093/brain/awr266

Anna M. Tedesco, Francesca R. Chiricozzi, Silvia Clausi, Michela Lupo, Marco Molinari, and Maria G. Leggio

Keywords: cerebellar role in non-motor functions, cerebellar cognitive affective syndrome, cerebellar damage, ataxia, cerebellum, cognition.

Pioglitazone and bladder cancer

In France, is currently running Friedreich's Ataxia clinical trial with Pioglitazone

The Lancet, Volume 378, Issue 9802, Pages 1543 - 1544, 29 October 2011

Dominique Hillaire-Buys a bEmail Address, Jean-Luc Faillie b c, Jean-Louis Montastruc d e

a CHRU Montpellier, Department of Medical Pharmacology and Toxicology, Lapeyronie Hospital, 34295 Montpellier, France
b Faculty of Medicine, University of Montpellier 1, Montpellier, France
c CHU Nîmes, Department of Epidemiology and Clinical Research, Nîmes University Hospital, Nîmes, France
d CHRU Toulouse, Department of Clinical Pharmacology, Toulouse University Hospital, Toulouse, France
e Department of Pharmacoepidemiology INSERM U1027, Faculty of Medicine, University of Toulouse, Toulouse, France

Keywords: Pioglitazone, peroxisome-proliferator-activated receptor (PPAR)agonist, oral hypoglycaemic drug, bladder cancer, US Food and Drug Administration (FDA), FDA Adverse Event Reporting System, European Medicines Agency.

Pioglitazone and bladder cancer

The Lancet, Volume 378, Issue 9802, Page 1544, 29 October 2011, doi:10.1016/S0140-6736(11)61663-2

Robert Elford Ryder
Department of Diabetes and Endocrinology, City Hospital, Birmingham B18 7QH, UK

Keywords: French and German regulatory bodies, risk of bladder cancer, European Medicines Agency (EMA), increased fractures, heart failure, type 2 diabetes, cardiovascular benefit.

"Despite the EMA's conclusion over bladder cancer risk, the overall risk—benefit balance remains strongly in favour of continued use of pioglitazone, especially in patients with ischaemic heart disease (but without heart failure) or stroke"

Pioglitazone and bladder cancer — Authors' reply

Erland Erdmann a, John A Dormandy b, Massimo Massi-Benedetti c, Robert Spanheimer

Monday, October 24, 2011

­Genes en lugar de fármacos

Interesting TV report on gene therapy (Spanish language)

Saturday, October 22, 2011

Hidden Administration of Drugs

Clinical Pharmacology & Therapeutics 90, 651-661 (November 2011) | doi:10.1038/clpt.2011.206

F Benedetti, E Carlino and A Pollo

Keywords: placebo-controlled trials, dummy treatment (placebo), psychological component, psychology and pharmacodynamics.

An unusual atrial tachycardia in a patient with Friedreich ataxia

Europace (2011) 13 (11): 1660-1661. doi: 10.1093/europace/eur156

Justin M.S. Lee1,*, Ian Turner1, Ajit Agarwal2 and Simon P. Fynn1
1Department of Cardiology, Papworth Hospital, Cambridge CB23 3RE, UK
2Department of Cardiology, West Suffolk Hospital, Bury St Edmunds, IP33 2QZ, UK

Keywords: atrial tachycardia, Friedreich ataxia, arrhythmi, linear ablation.