Characterizing gait, locomotor status, and disease severity in children and adolescents with friedreich ataxia.

J Neurol Phys Ther. 2009 Sep;33(3):144-9.

Croarkin E, Maring J, Pfalzer L, Harris-Love M, Siegel K, Diprospero N.
National Institutes of Health (E.C., K.S.), Bethesda, MD; George Washington University, Physical Therapy Program (J.M., M.H.L.), Washington, DC; University of Michigan School of Health Related Professions and Studies (L.P.), Ann Arbor, Michigan; and National Institute of Neurological Disorders and Stroke (N.D.P.), Bethesda, Maryland.

KEYWORDS: gait parameters, children, adolescents, Friedreich ataxia (FA), disease severity, Friedreich Ataxia Rating Scale (FARS), walk.

Mystery About Proteins That Package The Genome Solved

Florida State University. "Mystery About Proteins That Package The Genome Solved." ScienceDaily 8 October 2009

Keywords: regulate gene expression, histones, packaging DNA, genes are turned on or off,

Molecular signatures of disease brain endothelia provide new sites for CNS-directed enzyme therapy

Nature Medicine 15, 1215 - 1218 (2009)
Published online: 13 September 2009 | doi:10.1038/nm.2025

Yong Hong Chen¹, Michael Chang² & Beverly L Davidson^1,3

1. Department of Internal Medicine, Iowa City, Iowa, USA.
2. Departments of Molecular Physiology & Biophysics, Iowa City, Iowa, USA.
3. Department of Neurology, University of Iowa, Iowa City, Iowa, USA.

Keywors: Brain vasculature,  neural cells, microvessel,  enzyme replacement therapy, central nervous system (CNS), endothelia,  neurons,  glia, adeno-associated virus (AAV),  biodistribution.

Glutaredoxins: roles in iron homeostasis

Trends in Biochemical Sciences
Article in Press,
 
Nicolas Rouhier1, , Jérémy Couturier1, Michael K. Johnson2 and Jean-Pierre Jacquot1


1Unité Mixte de Recherches 1136 INRA Nancy University, Interactions Arbres Microorganismes, IFR 110 EFABA, Faculté des Sciences, BP 239 54506, Vandoeuvre Cedex, France
2Department of Chemistry and Center for Metalloenzyme Studies, University of Georgia, Athens, GA 30602, USA


Available online 5 October 2009.

Mitochondria - A neglected drug target.

Curr Opin Investig Drugs. 2009 Oct;10(10):1022-4.Links

Murphy MP.

Medical Research Council Mitochondrial Biology Unit, Wellcome Trust/MRC Building, Hills Road, Cambridge, CB2 0XY, UK. mpm@mrc-mbu.cam.ac.uk.

No Link Between Suicide and Stop-Smoking Drugs

By Kristina Fiore, Staff Writer, MedPage Today
Published: October 02, 2009
Reviewed by Robert Jasmer, MD; Associate Clinical Professor of Medicine, University of California, San Francisco. 

Keywords:  varenicline (Chantix)

Diverse effects in Friedreich's ataxia place PGC-1alpha center-stage.

Clin Genet. 2009 Oct;76(4):345-7
Metzler M.

Department of Medical Genetics, Centre for Molecular Medicine and Therapeutics, Child and Family Research Institute, Vancouver, BC, Canada. martina@cmmt.ubc.ca

Biophysical Characterization of the Iron in Mitochondria from Atm1p-Depleted Saccharomyces cerevisiae

Biochemistry, Article ASAP DOI: 10.1021/bi901110n
Publication Date (Web): September 17, 2009
 
Ren Miao, Hansoo Kim, Uma Mahendra Kumar Koppolu, E. Ann Ellis, Robert A. Scott and Paul A. Lindahl*

‡ Department of Chemistry § Microscopy and Imaging Center,
Department of Biochemistry and Biophysics
Texas A&M University, College Station, Texas 77843-3255
Department of Chemistry, University of Georgia, Athens, Georgia 30602-2556

Keywords: Atm1p, ABC transporter, mitochondrial inner membrane, cytosol, cellular iron metabolism, cytosolic Fe/S cluster assembly, mitochondrial Fe/S cluster assembly,  Mssbauer spectroscopy, EPR, electronic absorption spectroscopy, X-ray absorption spectroscopy, and electron microscopy, aerobically grown cells, iron(III) phosphate nanoparticles,  yeast frataxin Yfh1p-deleted, yeast ferredoxin Yah1p-depleted,  Atm1p-depleted, nonheme Fe(II) ions, oxidative damage,  cytosolic Fe/S cluster assembly,  oxygen.

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.