Wednesday, October 14, 2009

A comparison of three measures of upper limb function in Friedreich ataxia.

J Neurol. 2009 Oct 13.
Corben LA, Tai G, Wilson C, Collins V, Churchyard AJ, Delatycki MB.
Bruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research Institute, Flemington Rd, Parkville, VIC, 3052, Australia.

Keywords: Friedreich Ataxia (FRDA), false negative results, upper limb function, the Nine Hole Peg Test (9HPT), Box and Blocks Test (BBT), Jebsen Taylor Hand Function Test (JTHFT),Friedreich Ataxia Functional Composite (FAFC) test, clinical studies, terapeutic trials, age at disease onset, disease duration, Friedreich Ataxia Rating Scale (FARS).

Tuesday, October 13, 2009

Antioxidants and other pharmacological treatments for Friedreich ataxia.

Cochrane Database Syst Rev. 2009 Oct 7;4:CD007791

Kearney M, Orrell RW, Fahey M, Pandolfo M.
General Practice, Irish College of General Practitioners, Dunlavin, County Wicklow, Ireland.

Keywords: Friedreich ataxia, autosomal recessive, neurological disorder, slurred speech, scoliosis, pes cavus, heart abnormalities, antioxidants, controlled trials, international Co-operative Ataxia Rating Scale (ICARS),left ventricular heart mass, magnetic resonance imaging, echocardiography, idebenone.

Lipid Oxidation and Peroxidation in CNS Health and Disease: From Molecular Mechanisms to Therapeutic Opportunities

Rao Muralikrishna Adibhatla, James Franklin Hatcher, Savita Khanna, Shrinivas K. Kulkarni, Pamela Maher, Cesare Mancuso, Stanley I. Rapoport, Mark Smith, Bobby Thomas, Tatsurou Yagami. Antioxidants & Redox Signaling. -Not available-, ahead of print. doi:10.1089/ars.2009.2668.

Department of Neurological Surgery, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin.Cardiovascular Research Center, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin.Neuroscience Training Program, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin. William S. Middleton Veterans Affairs Hospital, Madison, Wisconsin.

Keywords: Reactive oxygen species (ROS), oxidative phosphorylation, NAD(P)H oxidases, arachidonic acid oxidative metabolism, endogenous antioxidant defenses, Nrf2/ARE, Oxidative stress, lipids, cell death, oxidized phospholipids, 4-hydroxynonenal, 4-oxo-2-nonenal, acrolein, neurodegenerative diseases (AD, ALS, bipolar disorder, epilepsy, Friedreich's ataxia, HD, MS, NBIA, NPC, PD, peroxisomal disorders, schizophrenia, Wallerian degeneration, Zellweger syndrome), CNS traumas (stroke, TBI, SCI), free iron, Fenton reaction, cholesterol, lipid oxidation/peroxidation.

Saturday, October 10, 2009

Risk/Benefit assessment, advantages over other drugs and targeting methods in the use of deferiprone as a pharmaceutical antioxidant in iron loading and non iron loading conditions.

Hemoglobin. 2009;33(5):386-97.

Kontoghiorghes GJ, Efstathiou A, Kleanthous M, Michaelides Y, Kolnagou A.

Postgraduate Research Institute of Science, Technology, Environment and Medicine, Limassol, Cyprus.

Keywords: oxidative stress, brain, heart, liver, kidneys, classical antioxidants, deferiprone, mobilizing labile iron and copper, free radicals, high therapeutic index, tissue penetration, rapid iron binding, clearance, iron complex, toxicity, cardiomyopathy, thalassemia, diabetic nephropathy, glomerulonephritis, kidney disease, Friedreich's Ataxia, Fanconi Anemia, deferoxamine (DFO), deferasirox (DFRA).

Thursday, October 8, 2009

Friedreich ataxia: a computational dynamic model of the key proteins involved in the yeast Fe–S cluster biogenesis

Friedreich ataxia: a computational dynamic model of the key proteins involved in the yeast Fe–S cluster biogenesis
New Biotechnology
Volume 25, Supplement 1, September 2009, Pages S342-S343
Abstracts of the 14th European Congress on BiotechnologyBarcelona, Spain 13–16 September, 2009

I. Amela1, J. Cedano1, P. Delicado2 and E. Querol1

1Universitat Autònoma de Barcelona, Bellatera - Barcelona, Spain

2Universitat Politècnica de Catalunya, Barcelona, Spain

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,

Wednesday, October 7, 2009

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 Davidson1,3

  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.

Sunday, October 4, 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.