Uses of the postural stability test for differential diagnosis of hereditary ataxias.

J Neurol Sci. 2012 Feb 13. http://dx.doi.org/10.1016/j.jns.2012.01.022.

J. Schwabova a, F. Zahalka c, V. Komarek b, T. Maly c, P. Hrasky c, T. Gryc c, O. Cakrt d, A. Zumrova b

a Department of Neurology, Charles University in Prague, 2nd Faculty of Medicine and the Motol University Hospital, V Úvalu 84, Prague 5, 150 06, Czech republic
b Department of Paediatric Neurology, Charles University in Prague, 2nd Faculty of Medicine and the Motol University Hospital, V Úvalu 84, Prague 5, 150 06, Czech republic
c Research Sport Center, Charles University in Prague, Faculty of Physical Education and Sport, Jose Martího 31, Prague 6, 162 52, Czech republic
d Department of Rehabilitation and Sport Medicine, Charles University in Prague, 2nd Faculty of Medicine and the Motol University Hospital, V Úvalu 84, Prague 5, 150 06, Czech republic

Keywords: Friedreich's ataxia (FRDA), spinocerebellar ataxia type 2 (SCA 2), posturographic examination, differential diagnosis.

The Autosomal Recessive Cerebellar Ataxias

N Engl J Med 2012; 366:636-646, February 16, 2012

Mathieu Anheim, M.D., Ph.D., Christine Tranchant, M.D., and Michel Koenig, M.D., Ph.D.

Review Article

Substantia nigra hypoechogenicity in Friedreich ataxia.

Mov Disord 2012 Feb; 27(2):332-3.DOI: 10.1002/mds.23989

Stockner H, Sojer M, Hering S, Nachbauer W, Seppi K, Schmidauer C, Poewe W, Boesch SM
Department of Neurology, Medical University Innsbruck, Innsbruck, Austria.

No abstract is available for this article.

Histone deacetylase inhibition activates transcription factor Nrf2 and protects against cerebral ischemic damage.

Free Radic Biol Med. Volume 52, Issue 5, 1 March 2012, Pages 928–936, http://dx.doi.org/10.1016/j.freeradbiomed.2011.12.006

Wang B, Zhu X, Kim Y, Li J, Huang S, Saleem S, Li RC, Xu Y, Dore S, Cao W.

Nanjing University Medical School, Jiangsu Key Lab of Molecular Medicine,
Nanjing, People's Republic of China; Department of Anesthesiology and Critical
Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MD 21205,
USA.

Keywords: histone deacetylase (HDAC), neuroprotection, multiple mechanisms, modulation of transcription, Nrf2, stroke, antioxidant-responsive genes, activation of Nrf2.

Cognitive function in Friedreich's ataxia patients in Ireland

J Neurol Neurosurg Psychiatry 2012;83:e1 doi:10.1136/jnnp-2011-301993.43

C Albertyn, N Austin, R P Murphy
Adelaide and Meath Hospital, Dublin, Ireland

Keywords: Friedreich's ataxia (FRDA), cognitive function, genetically confirmed FRDA patients, age of onset, duration of symptoms, Friedreich's ataxia rating scale (FARS) score, neuropsychological test battery assessed immediate and delayed verbal memory, visuospatial perception, confrontational naming, non-verbal memory, attention and concentration, premorbid intellect, executive function, cognitive speed.

Determination of Idebenone in Plasma by HPLC with Post -Column Fluorescence Derivatization using 2-Cyanoacetamide

Chem Pharm Bull, Regular Article, Analytical Chemistry

Yukio NOHARA a, Junko SUZUKI b, Yoji YAMAZAKI a, Hiroaki KUBO a.
a Faculty of Pharmacy, Iwaki Meisei University, Iwaki, Fukushima 970-8551, Japan
b School of Pharmacy, Kitasato University, Minato-ku, Tokyo 108-8641, Japan

Keywords: Idebenone, Friedreich’s ataxia, 2-cyanoacetamide, fluorescence, HPLC with post-column fluorescence derivatization, human blood plasma, finger prick.

New website: NIH Clinical Research Trials and You

The National Institutes of Health has created a new website, NIH Clinical Research Trials and You to help people learn more about clinical trials, why they matter, and how to participate.

GO to the new website

A Drosophila Model of Friedreich's Ataxia and Autophagic Heart Disease.

Wayne State Univ, ?.

Luan Wang. Inst Environmental Hlth Sci, Wayne State Univ, Detroit, MI.

Keywords: Friedreich’s Ataxia (FRDA), Drosophila model for FRDA, UAS-Gal4 system, heart (tinman), neurons (elav), whole body (actin and daG32), reactive oxygen species (ROS) reagent, oxidative stress, autophagy activity.

"This suggests that there is a threshold for frataxin level to maintain the normal function."

OX1 for Friedreich's Ataxia likely would enter Phase 2 clinical trials early in 2013

NEW YORK, Feb.2, 2012 /PRNewswire/ --Intellect Neurosciences, Inc. (OTCBB:ILNS), a biopharmaceutical company engaged in the discovery and development of disease-modifying therapeutic agents for the treatment of Alzheimer's and other neurological diseases today issued the following Letter to Shareholders from Dr. Daniel Chain, Chairman and CEO.

ViroPharma's CEO, spoke enthusiastically about his company's plans to develop OX1 for Friedreich's Ataxia, indicating OX1 likely would enter Phase 2 clinical trials early in 2013.

Drugs and mitochondrial diseases: 40 queries and answers

Expert Opinion on Pharmacotherapy 0 0:0, 1-17, Posted online on January 31, 2012. (doi:10.1517/14656566.2012.657177)

Michelangelo Mancuso †1 MD PhD, Daniele Orsucci 1, Massimiliano Filosto 2, Costanza Simoncini 1 & Gabriele Siciliano 1
1 University of Pisa, Neurological Clinic, Department of Neuroscience,
Via Roma 67, 56126 Pisa, Italy
2 University of Brescia, Italy

Keywords
carnitine, coenzyme Q10, creatine, mitochondria, mitochondrial diseases, mtDNA, riboflavin, Mitochondrial disorders, oxidative phosphorylation, respiratory chain cofactors, antioxidants, lactic acidosis, basic and clinical literature.