Ataxia with vitamin E deficiency in southeast Norway, case report.

Koht J, Bjørnarå KA, Jørum E, Tallaksen CM.
Faculty of Medicine, University of Oslo, Oslo, Norway.

Keywords: AVED, Friedreich's ataxia, Frataxin gene, unknown prevalence, Norway, preschool age, neurological examination, re-evaluation, re-examination, neuropathy. Vitamin E, heterozygous mutation, p.A120T, p.R134X, alpha-tocopherol transport protein gene, chromosome 8q13, treatment available,

Nitric oxide and frataxin: two players contributing to maintain cellular iron homeostasis.

Ann Bot (Lond). 2009 Jun 25

Ramirez L, Zabaleta EJ, Lamattina L.
Instituto de Investigaciones Biológicas, Facultad de Ciencias Exactas y Naturales, CONICET-Universidad Nacional de Mar del Plata, CC 1245, (7600) Mar del Plata, Argentina.

Keywords: PLANTS, animals, bacteria, Nitric oxide, iron metabolism, Frataxin, mitochondrial iron homeostasis, frataxin knock-down Arabidopsis thaliana mutants.

NITRIC OXIDE IN CELL SURVIVAL: A JANUS MOLECULE.

Antioxid Redox Signal. 2009 Jun 26

Calabrese V, Cornelius C, Rizzarelli E, Owen JB, Dinkova-Kostova AT, Butterfield DA.
University of Catania, Department of Chemistry, Catania, Italy; calabres@unict.it.

Keywors: Nitric oxide, nervous system, synaptic activity, neural plasticity, memory function, cysteine residues , neuroinflammation, neurodegeneration, Alzheimer's disease, amyothrophic lateral sclerosis, Parkinson's disease, multiple sclerosis, Friedreich's ataxia, Huntington, glutathione, nitrosative stress, redox signaling.

FEDER presenta la Primera Guía Psicológica de Afectados por ER

La organización y gracias al apoyo de Merck Serono ha desarrollado esta publicación que tiene por objetivo ofrecer herramientas de intervención con familias y afectados

Comparison of three clinical rating scales in Friedreich ataxia (FRDA)

Katrin Bürk, MD 1 2 *, Ulrike Mälzig, MD 1, Stefanie Wolf, PhD 1, Suzette Heck, MD 3, Konstantinos Dimitriadis, MD 3, Tanja Schmitz-Hübsch, MD 4, Sascha Hering, MD 5, Tobias M. Lindig, MD 6, Verena Haug, MD 7, Dagmar Timmann, MD 8, Ingrid Degen, MD 9, Bernd Kruse, MD 10, Jan-Markus Dörr, MD 11, Susanne Ratzka, MD 1, Anja Ivo, MD 4, Ludger Schöls, MD 6, Sylvia Boesch, MD 5, Thomas Klockgether, MD 4, Thomas Klopstock, MD 3, Jörg B. Schulz, MD 1

1Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University of Göttingen, Göttingen, Germany 2Department of Neurology, University of Marburg, Marburg, Germany 3Department of Neurology, University of Munich, Munich, Germany 4Department of Neurology, University of Bonn, Bonn, Germany 5Department of Neurology, University of Innsbruck, Innsbruck, Austria 6Department of Neurodegeneration and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany 7Department of Neuropediatrics, University of Freiburg, Freiburg, Germany 8Department of Neurology, University of Essen, Essen, Germany 9Department of Neuropediatrics, Sankt Elisabeth Krankenhaus, Neuwied, Germany 10Department of Neuropediatrics, University of Hamburg, Hamburg, Germany 11Department of Neurology, Helios-Klinikum, Berlin, Germany

email: Katrin Bürk (buerk@ngi.de)

^*Correspondence to Katrin Bürk, Department of Neurodegeneration and Restorative Research, Center of Molecular Physiology of the Brain and Center of Neurological Medicine, University of Göttingen, Waldweg 33, Göttingen D-37073, Germany

Keywords: Friedreich ataxia • clinical rating scales • validation • SARA • ICARS • FARS

Development of a brief ataxia rating scale (BARS) based on a modified form of the ICARS

Jeremy D. Schmahmann, MD *, Raquel Gardner, MD, Jason MacMore, BA, Mark G. Vangel, PhD

Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA

email: Jeremy D. Schmahmann (jschmahmann@partners.org)

^*Correspondence to Jeremy D. Schmahmann, Department of Neurology, Massachusetts General Hospital, Suite 340, Charles River Plaza South, 55 Fruit Street, Boston, Massachusetts 02114

Keywords

ataxia • dysmetria • rating scale • assessment • cerebellum

PGC-1[alpha] AND PGC-1[beta] regulate mitochondrial density in neurons.

J Biol Chem. 2009 Jun 19.

Przemyslaw W, Vaarmann A, Choubey V, Safiulina D, Liiv J, Kuum M, Kaasik A.
Department of Pharmacology, University of Tartu, Estonia.

Link to full text: http://www.jbc.org/cgi/reprint/M109.018911v1?view=long&pmid=19542216

Keywords: PGC-1[alpha], PGC-1[beta, peroxisome-proliferator-activated receptor gamma coactivator-1a, mitochondrial biogenesis , cellular energy metabolism , muscle, liver, neurons, activates transcriptional activity of PGC-1[alpha] in neurons, compensate neuronal mitochondrial loss , treating neurodegenerative diseases , mitochondrial dysfunction , oxidative damage.

Antidepressant directly stimulates brain growth factor receptors

PHYSORG.COM, June 25th, 2009

The widely used antidepressant and pain medication amitriptyline--but not other closely related drugs -- can impersonate the brain's own growth factors, researchers at Emory University School of Medicine have shown.

Other sources:
http://www.sciencedaily.com/releases/2009/06/090625133059.htm
http://www.eurekalert.org/pub_releases/2009-06/eu-ads062209.php

Creating zinc monkey wrenches in the treatment of epigenetic disorders.

Curr Opin Chem Biol. 2009 Jun 19

Kalin JH, Butler KV, Kozikowski AP.University of Illinois at Chicago, Department of Medicinal Chemistry, 833 South Wood Street, Chicago, IL 60612, USA

Keywords: suberoylanilide hydroxamic, histone deacetylase inhibitors, HDACIs, neurodegenerative disorders, cancers, bipolar disorder , depression, Friedreich's ataxia, Huntington's


Comment: Revision of the HDACIs, an emerging new generation of drugs for diseases difficult to cure, in which we have great hope.

PGC-1beta: A Co-activator That Sets the Tone for Both Basal and Stress-Stimulated Mitochondrial Activity.

Adv Exp Med Biol. 2009;646:133-9.

Lelliott CJ, Vidal-Puig A.Department of Clinical Biochemistry, University of Cambridge, Addenbrooke's Hospital, Hills Road, Cambridge, CB2 2QR, UK, christopher.lelliott@astrazeneca.com.

Keywords: PGC, transcriptional coactivators , PGC-1beta, mouse PGC1betaKO, brown adipose tissue (BAT), translates into mitochondrial dysfunction , handle physiological stresses.