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.

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;

Structure and Dynamics of the Iron−Sulfur Cluster Assembly Scaffold Protein IscU and Its Interaction with the Cochaperone HscB

Jin Hae Kim§, Anna K. Fzry, Marco Tonelli, Dennis T. Ta@, William M. Westler, Larry E. Vickery@ and John L. Markley*§

§Biophysics Graduate ProgramDepartment of Biochemistry,National Magnetic Resonance Facility at MadisonUniversity of Wisconsin, Madison, Wisconsin 53706@Department of Physiology and Biophysics, University of California, Irvine, California 92697

Biochemistry, Article ASAP

DOI: 10.1021/bi9002277

Publication Date (Web): June 3, 2009

Copyright © 2009 American Chemical Society

*To whom correspondence should be addressed. Phone: (608) 263-9349. Fax: (608) 262-3759. E-mail: markley@nmrfam.wisc.edu.

CeNeRx BioPharma Obtains Rights To Novel Drug Candidate For Prevention And Treatment Of Neurodegeneration Disorders

CeNeRx BioPharma Obtains Rights To Novel Drug Candidate For Prevention And Treatment Of Neurodegeneration Disorders

Medical News Today, Article Date: 17 Jun 2009 - 3:00 PDT

Full Text: http://www.medicalnewstoday.com/articles/154197.php

Keywords: central nervous system , neuropathies, neurodegenerative disorders, CXB909, nerve growth factor (NGF), Phase l trial , blood brain barrier.

Press realease of CeNeRx Biopharma: http://cenerx.com/News/releases/16a-JUN-09.pdf

DRG-targeted helper-dependent adenoviruses mediate selective gene delivery for therapeutic rescue of sensory neuronopathies in mice

J. Clin. Invest. doi:10.1172/JCI39038

Copyright © 2009, The American Society for Clinical Investigation



DRG-targeted helper-dependent adenoviruses mediate selective gene delivery for therapeutic rescue of sensory neuronopathies in mice


Tomoya Terashima1, Kazuhiro Oka1,2, Angelika B. Kritz3, Hideto Kojima2, Andrew H. Baker3 and Lawrence Chan1,2
1Department of Medicine and 2Department of Molecular and Cellular Biology, Baylor College of Medicine, Houston, Texas, USA. 3British Heart Foundation Glasgow Cardiovascular Research Centre, University of Glasgow, Glasgow, United Kingdom.
Address correspondence to: Lawrence Chan, Department of Medicine, Baylor College of Medicine, One Baylor Plaza, Houston, Texas 77030, USA. Phone: (713) 798-4478; Fax: (713) 798-8764; E-mail: lchan@bcm.tmc.edu.



Published June 15, 2009



Full text: http://www.jci.org/articles/view/39038



Keywords: sensory neuronopathies, painful , DRG, therapeutic genes , dorsal root ganglion , adenoviruses, mice.

Therapeutic Delivery Of A Gene To Dysfunctional Nerves

Therapeutic Delivery Of A Gene To Dysfunctional Nerves

ScienceDaily (June 15, 2009) — In many sensory neuronopathies, painful conditions affecting sensory nerves outside the brain and spinal cord, the affected nerves are in a region known as the DRG. These conditions are difficult to treat. However, researchers have now developed an approach to target therapeutic genes to DRG nerves, and used it to reduce sensory nerve dysfunction in a mouse model of Sandhoff disease, an inherited condition in which many nerves, including those in the DRG, are affected.

Full Text http://www.sciencedaily.com/releases/2009/06/090615171509.htm

Keywords: sensory neuronopathies, painful , DRG, therapeutic genes , dorsal root ganglion , adenoviruses, mice.

OPEN ACCES

PLoS ONE, Full text

Fine Mapping of Gene Regions Regulating Neurodegeneration

Maria Swanberg^1,2, Karin Harnesk^1#, Mikael Ström^1#, Margarita Diez¹, Olle Lidman¹, Fredrik Piehl^1*

1 Department of Clinical Neuroscience, Karolinska Institutet, Karolinska Hospital, Stockholm, Sweden, 2 Department of Clinical Sciences, Malmö, Lund University, Malmö, Sweden

Abstract

Background

Damage to nerve cells and axons leading to neurodegeneration is a characteristic feature of many neurological diseases. The degree of genetic influence on susceptibility to axotomy-induced neuronal death has so far been unknown. We have examined two gene regions, Vra1 and Vra2, previously linked to nerve cell loss after ventral root avulsion in a rat F2 intercross between the DA and PVG inbred rat strains.

Methodology/Principal Findings

In this study, we use two generations (G8 and G10 cohorts) of an advanced intercross line between DA and PVG^av1 to reproduce linkage to Vra1 and to fine-map this region. By isolating the effect from Vra1 in congenic strains, we demonstrate that Vra1 significantly regulates the loss of motoneurons after avulsion. The regulatory effect mediated by Vra1 thus resides in a congenic fragment of 9 megabases. Furthermore, we have used the advanced intercross lines to give more support to Vra2, originally detected as a suggestive QTL.

Conclusions/Significance

The results demonstrated here show that naturally occurring allelic variations affect susceptibility to axotomy-induced nerve cell death. Vra1 and Vra2 represent the first quantitative trait loci regulating this phenotype that are characterized and fine mapped in an advanced intercross line. In addition, congenic strains provide experimental evidence for the Vra1 effect on the extent of injury-induced neurodegeneration. Identification of the underlying genetic variations will increase our understanding of the regulation and mechanisms of neurodegeneration.

Citation: Swanberg M, Harnesk K, Ström M, Diez M, Lidman O, et al. (2009) Fine Mapping of Gene Regions Regulating Neurodegeneration. PLoS ONE 4(6): e5906. doi:10.1371/journal.pone.0005906

Editor: Rafael Linden, Universidade Federal do Rio de Janeiro (UFRJ), Instituto de Biofísica da UFRJ, Brazil

Received: March 19, 2009; Accepted: May 19, 2009; Published: June 15, 2009

Copyright: © 2009 Swanberg et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Funding: This study was supported by the 6th Framework Program of the European Union, NeuroproMiSe, LSHM-CT-2005-018637 and EURATools, LSHG-CT-2005019015, the Swedish Research Council, Wadsworth Foundation, the Swedish Society for Medical Research, the Swedish Brain Foundation, the Nils and Bibbi Jenssens Foundation, the Montel Williams MS Foundation, the GV80 Foundation, the Soderberg Foundation, and the Swedish Association of Persons with Neurological Disabilities. The funders had no role in study design, data collection or analysis, manuscript preparation or decision to publish.

Competing interests: The authors have declared that no competing interests exist.

* E-mail: fredrik.piehl@ki.se

# These authors contributed equally to this work.

Two Signals -- From Within And Out Of Cell -- Specify Motor Neuron Differentiation

ScienceDaily (June 10, 2009) -Science News

Two Signals -- From Within And Out Of Cell -- Specify Motor Neuron Differentiation

ScienceDaily (June 10, 2009) — Two signals – an external one from retinoic acid and an internal one from the transcription factor Neurogenin2 – cooperate to activate chromatin (the basic material of chromosomes) and help determine that certain nerve progenitor cells become motor neurons, said researchers from Baylor College of Medicine in a report in the current issue of the journal Neuron.

Full text: http://www.sciencedaily.com/releases/2009/06/090610124411.htm

Keywords: motor neurons , stem cells, Neurogenin2, retinoic acid , histone acetyltransferase , histones , transcription , nerve progenitor cells, muscular dystrophies

Oligomeric yeast frataxin drives assembly of core machinery for mitochondrial iron-sulfur cluster synthesis.

J Biol Chem. 2009 Jun 2.

Oligomeric yeast frataxin drives assembly of core machinery for mitochondrial iron-sulfur cluster synthesis.

Li H, Gakh O, Smith DY 4th, Isaya G.

Mayo Clinic, College of Medicine, United States.

Mitochondrial biosynthesis of iron-sulfur clusters (ISC)(1) is a vital process involving the delivery of elemental iron and sulfur to a scaffold protein .....

Keywords: iron-sulfur clusters, iron, sulfur, Yfh1, Nfs1-Isd11, Isu1, oligomerization, oxidation, mechanism, mitochondrial ISC synthesis..

Neural stem cell boosting chemical that generate new neurons - received U.S. patent allowance

"Stem Cell Research Blog" 06 Jun 2009 12:43 pm

Neural-stem-cell-boosting-chemical-that-generate-new-neurons-received-us-patent-allowance

The U.S. Patent and Trademark Office (USPTO) have delivered a notice of allowance to Neuralstem, Inc. for a patent on four new chemicals that can boost the generation of new neurons. .....

Keywords: Neuralstem Inc, patent, neurons, neurogenic activity, drugs, human neural stem cells, hippocampus.

Subjective Improvement in Proprioception in 2 Patients With Atypical Friedreich Ataxia Treated With Varenicline (Chantix)

Journal of Clinical Neuromuscular Disease:
June 2009 - Volume 10 - Issue 4 - pp 191-193
doi: 10.1097/CND.0b013e3181910074

Subjective Improvement in Proprioception in 2 Patients With Atypical Friedreich Ataxia Treated With Varenicline (Chantix)
Zesiewicz, Theresa A MD, FAAN; Sullivan, Kelly L MSPH; Gooch, Clifton L MD; Lynch, David R MD, PhD

Keywords: Friedreich ataxia , proprioceptive improvements , varenicline (Chantix), smoking cessation.

Medical News Today: Article Date: 04 Jun 2009 - 7:00 PDT
Source: Nancy Ross-Flanigan. University of Michigan

Small Molecules Mimic Natural Gene Regulators

In the quest for new approaches to treating and preventing disease, one appealing route involves turning genes .......

.../...
These molecules can help scientists probe the transcription process and perhaps eventually be used to correct diseases that result from errors in gene regulation.
.../...

Keywords: turning genes, cancer, diabetes, gene expression, transcriptional activators , CBP.

Original work:( http://pubs.acs.org/doi/abs/10.1021/cb900028j?prevSearch=David+Wemmer&searchHistoryKey= )

Amphipathic Small Molecules Mimic the Binding Mode and Function of Endogenous Transcription Factors

Sara J. Buhrlage†, Caleb A. Bates‡, Steven P. Rowe†, Aaron R. Minter†, Brian B. Brennan†, Chinmay Y. Majmudar†, David E. Wemmer§, Hashim Al-Hashimi† and Anna K. Mapp†‡¶*

† Department of Chemistry ‡ Department of Medicinal Chemistry Department of Biophysics ¶ Program in Chemical Biology, University of Michigan, Ann Arbor, Michigan, 48109 § Department of Chemistry, University of California, Berkeley, California 94720

ACS Chem. Biol., 2009, 4 (5), pp 335–344

DOI: 10.1021/cb900028j

Publication Date (Web): April 6, 2009

Copyright © 2009 American Chemical Society

Friedreich ataxia (fa) associated with diabetes mellitus type 1 and hyperthrophic cardiomyopathy.

Friedreich ataxia (fa) associated with diabetes mellitus type 1 and hyperthrophic cardiomyopathy.

Bosn J Basic Med Sci. 2009 May;9(2):107-10.

Gucev Z, Tasic V, Jancevska A, Popjordanova N, Koceva S, Kuturec M, Sabolic V.
Faculty of Medicine Skopje, St. Cyril and Methodius University, 50 Divizija BB, 1000 Skopje, Macedonia.

Progressive signs of ataxia in a eight years old girl prompted neurological investigation. The girl had unstable gait .....

Keywords: Friedreich ataxia, dysarthria, pes cavus, positive Babinski sign, scoliosis, diabetic ketoacidosis, GAA trinucleotide repeat, sinus bradycardia, hyperthrophic cardiomyopathy, beta blocker, insulin.

Prospects for introducing deferiprone as potent pharmaceutical antioxidant.

Prospects for introducing deferiprone as potent pharmaceutical antioxidant.

Front Biosci (Elite Ed). 2009 Jun 1;1:161-178.

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

Free radical formation is primarily initiated from metal catalytic centers involving iron and copper. Under certain conditions, free radical reactions .......

Keywords: Free radical, iron, copper, oxidative stress, antioxidants, chelators, Deferiprone, cardiomyopathy, thalassaemia, Friedreich ataxia.