Saturday, September 26, 2009

Chemical Probes Identify a Role for Histone Deacetylase 3 in Friedreich's Ataxia Gene Silencing

Chemistry & Biology, Volume 16, Issue 9, 980-989, 25 September 2009
Chunping Xu1, Elisabetta Soragni1, C. James Chou1, David Herman1, Heather L. Plasterer2, James R. Rusche2 and Joel M. Gottesfeld1,

1 Department of Molecular Biology, The Scripps Research Institute, La Jolla, CA 92037, USA
2 Repligen Corporation, Waltham, MA 02453, USA

Keywords: pimelic diphenylamide histone deacetylase (HDAC) inhibitors, Friedreich's ataxia (FRDA), Huntington's disease, HDAC3.

Neurophysiological evaluation in children with Friedreich's ataxia.

Early Hum Dev. 2009 Sep 21.

Sival DA, du Marchie Sarvaas GJ, Brouwer OF, Uges DR, Verschuuren-Bemelmans CC, Maurits NM, Brunt ER, van der Hoeven JH.


Department of Pediatrics, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, The Netherlands.

Keywords:  Friedreich's ataxia (FRDA children), ICARS, idebenone treatment, longitudinal neurophysiological parameters,   two-year study,  period, cardiomyopathy (6-18years), sensory evoked potentials (SEPs), F response, peripheral nerve conduction , dynamometry, SEPs , peroneal nerve conduction velocity,  not substantiate neurologic improvement.