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.

Neuronal metabolism: A question of balance

Katherine Whalley

Nature Reviews Neuroscience 10, 472 - 473 (2009) | doi:10.1038/nrn2669.

Mitochondrial membrane permeabilization in neuronal injury

Nature Reviews Neuroscience 10, 481-494 (July 2009) | doi:10.1038/nrn2665

Lorenzo Galluzzi^1,2, Klas Blomgren^3,4 & Guido Kroemer^1,2

1. INSERM, U848, Institut Gustave Roussy, PR1, 39 rue Camille Desmoulins, F-94805 Villejuif, France.
2. Université Paris-Sud XI, F-94805 Villejuif, France.
3. Center for Brain Repair and Rehabilitation, Institute of Neuroscience and Physiology, University of Gothenburg, SE-405 30 Gothenburg, Sweden.
4. Department of Pediatric Oncology, The Queen Silvia Children's Hospital, SE-416 85 Gothenburg, Sweden.

Correspondence to: Guido Kroemer^1,2 Email: kroemer@orange.fr

Autosomal Recessive Ataxia Caused by Three Distinct Gene Defects in a Single Consanguineous Family

Authors: Yosr Bouhlal ^a; Mourad Zouari ^a; Mounir Kefi ^a; Christiane Ben Hamida ^a; Fayal Hentati ^a; Rim Amouri ^a

Affiliation:

a Laboratoire de Neurobiologie Molculaire et de Neuropathologie, Institut National de Neurologie, Tunisia

DOI: 10.1080/01677060802025233

Published in: Journal of Neurogenetics, Volume 22, Issue 2 June 2008 , pages 139 - 148

Subjects: Cell Biology; Genetics; Molecular Biology; Neuroscience;