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.

SCA-LSVD: a repeat-oriented locus-specific variation database for genotype to phenotype correlations in spinocerebellar ataxias.

Hum Mutat. 2009 Jul;30(7):1037-42
Faruq M, Scaria V, Singh I, Tyagi S, Srivastava AK, Mukerji M.


Genomics and Molecular Medicine, Institute of Genomics and Integrative Biology-Council of Scientific and Industrial Research (IGIB-CSIR), Delhi, India.

Keyword: Repeat expansion, autosomal dominant cerebellar ataxias (ADCAs), spinocerebellar ataxias (SCAs),   clinical classification,  prevalence, phenotypic expression,  ethnic groups, SCA-locus-specific variation database (LSVD),  SCA1, SCA 2, SCA 3, SCA 6, SCA 7, SCA 8, SCA 12, SCA 17, Friedreich's ataxia [FRDA], dentatorubral-pallidoluysian atrophy [DRPLA]),  Leiden Open (source) Variation Database (LOVD) software,  clinical features, HGVS Nomenclature guidelines.

High frequency of Friedreich's ataxia carriers in the Paphos district of Cyprus.

Acta Myol. 2009 Jul;28(1):24-6.

Zamba-Papanicolaou E, Koutsou P, Daiou C, Gaglia E, Georghiou A, Christodoulou K.
The Cyprus Institute of Neurology and Genetics, Nicosia, Cyprus.

Keyword: Friedreich's ataxia, Paphos, Cyprus,  GAA triplet repeat, frataxin gene,  carrier frequency,  organized prevention programme.

Mitochondrial Superoxide Radicals Differentially Affect Muscle Activity and Neural Function

Genetics, Vol. 183, 175-184, September 2009

Tanja Godenschwege*,1, Renée Forde,1, Claudette P. Davis, Anirban Paul,2, Kristopher Beckwith and Atanu Duttaroy,3


* Department of Biological Sciences, Florida Atlantic University, Boca Raton, Florida 33431 and Biology Department, Howard University, Washington, DC 20059

Disruption to higher order processes in Friedreich ataxia

doi:10.1016/j.neuropsychologia.2009.09.009
Received 1 April 2009; revised 21 July 2009; accepted 11 September 2009. 

Available online 17 September 2009.

Joanne Fielding^{a, b, c,}, Louise Corben^{d, e}, Phillip Cremer^f, Lynette Millist^c, Owen White^{b, c} and Martin Delatycki^{d, g}

^aCentre for Neuroscience, University of Melbourne, Parkville, Victoria, Australia
^bCentre for Developmental Psychiatry and Psychology, School of Psychology, Psychiatry, and Psychological Medicine, Monash University, Clayton, Victoria, Australia
^cDepartment of Neurology, Royal Melbourne Hospital, Parkville, Victoria, Australia
^dBruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research Institute, Victoria, Australia
^eExperimental Neuropsychology Research Unit, School of Psychology, Psychiatry, and Psychological Medicine, Monash University, Clayton, Victoria, Australia
^fRoyal North Shore Hospital, N.S.W., Australia
^gDepartment of Paediatrics, University of Melbourne, Victoria, Australia

 Keywords: Friedreich ataxia (FRDA); cognition; saccades; cerebellum