HDAC6 is a target for protection and regeneration following injury in the nervous system.

Proc Natl Acad Sci U S A. 2009 Nov 17;106(46):19599-604. Epub 2009 Nov 2.

Rivieccio MA, Brochier C, Willis DE, Walker BA, D'Annibale MA, McLaughlin K, Siddiq A, Kozikowski AP, Jaffrey SR, Twiss JL, Ratan RR, Langley B.
Burke Medical Research Institute, 785 Mamaroneck Avenue, White Plains, NY 10605, USA.

Keywords:Central nervous system (CNS) trauma,, neuronal degeneration, axonal degeneration, toxicity, anticancer properties, neuronal protection against oxidative stress,  promotes neurite growth,  HDAC6.


 

Metal chelators coupled with nanoparticles as potential therapeutic agents for Alzheimer's disease.

J Nanoneurosci. 2009 Jun 1;1(1):42-55.

Liu G, Men P, Perry G, Smith MA.

Department of Radiology, University of Utah, Salt Lake City, UT 84108, USA.

Keywords: Alzheimer's disease (AD), blood-brain barrier,  nanoparticle-mediated drug delivery,  chelation agents,  iron-associated neurodegenerative diseases, Friedreich's ataxia, Parkinson's disease, Huntington's disease,  Hallervorden-Spatz Syndrome, nanoparticle technology, very early stages of development.

Clinical aspects of coenzyme Q(10): An update.

Nutrition. 2009 Nov 20.

Littarru GP, Tiano L.
Department of Biochemistry, Biology and Genetics, Polytechnic University of the Marche, Ancona, Italy.

Keywords:  Q(10) (CoQ(10)),  in mitochondrial bioenergetics,  antioxidant properties, clinical applications, cardiovascular disease, physical exercise, mitochondrial myopathies, Parkinson's and Huntington's diseases,  Friedreich's ataxia,  preeclampsia in pregnancy. headache symptoms, pediatric and adolescent populations.

A theoretical analysis on characteristics of protein structures induced by cold denaturation

J. Chem. Phys. 131, 205102 (2009); doi:10.1063/1.3265985

Published 24 November 2009

Hiraku Oshima, Takashi Yoshidome, Ken-ichi Amano, and Masahiro Kinoshita

Institute of Advanced Energy, Kyoto University, Uji, Kyoto 611-0011, Japan

Keywords:  frataxin, cold denaturation,  morphometric approach, entropy change,  unfolded structures.

A Diet Enriched in Polyphenols and Polyunsaturated Fatty Acids, LMN Diet, Induces Neurogenesis in the Subventricular Zone and Hippocampus of Adult Mouse Brain

Journal of Alzheimer's Disease, IOS Press, DOI 10.3233/JAD-2009-1188
Authors


Tony Valente1, Juan Hidalgo2, Irene Bolea1, Bartolomé Ramirez3, Neus Anglés3, Jordi Reguant3, José Ramón Morelló3, Cristina Gutiérrez1, Mercè Boada4, Mercedes Unzeta1

1Departament de Bioquimica i Biologia Molecular, Institut de Neurociències, Facultat de Medicina, Torre M2, Universitat Autònoma de Barcelona, Bellaterra, Barcelona, Spain
2Institut de Neurociències, and Departamento de Biología Celular, Fisiologia, e Inmunología, Facultat de Biociències, Universitat Autònoma de Barcelona, Bellaterra, Barcelona, Spain
3La Morella Nuts SA, Reus, Tarragona, Spain
Fundació ACE, Institut Català de Neurociències Aplicades, Barcelona, Spain

Keywords: 129S1/SvImJ mice, adult neurogenesis, diet, hippocampus, olfactory bulb, polyphenols, polyunsaturated fatty acids, several neurodegenerative pathologies, potent antioxidant effect, neurodegenerative diseases.

Full text:  http://iospress.metapress.com/content/91261l2n31q4q797/fulltext.pdf

Polyphenols and Polyunsaturated Fatty Acids Boost the Birth of New Neurons, Study Finds

ScienceDaily (Nov. 24, 2009) — Universitat Autònoma de Barcelona (UAB) researchers have confirmed that a diet rich in polyphenols and polyunsaturated fatty acids, patented as an LMN diet, helps boost the production of the brain's stem cells -neurogenesis- and strengthens their differentiation in different types of neuron cells. read more....

Histone modifications are specifically relocated during gene activation and nuclear differentiation

OPEN ACCESS

Katharina S Heyse , Susanne E Weber and Hans Joachim Lipps

BMC Genomics 2009, 10:554doi:10.1186/1471-2164-10-554
Published: 24 November 2009

Abstract (provisional)

Background
Post-translational histone modifications (PTMs) and their specific distribution on genes play a crucial role in the control of gene expression, but the regulation of their dynamics upon gene activation and differentiation is still poorly understood. Here, we exploit the unique genome organization of ciliates to analyse PTM dynamics during gene activation in the differentiated cell and during nuclear differentiation. In the macronucleus of these cells the DNA is organized into nanochromosomes which represent independent functional units. Therefore ciliated protozoa represent a simplistic model system to analyse the relevance of histone modifications and their localization for gene expression and differentiation.

Results
We analysed the distribution of three PTMs on six individual nanochromosomes, two of which are silenced in the vegetative cell and only activated during sexual reproduction. We show that a specific relocation of these PTMs correlates with gene activation. Moreover, macronuclear-destined sequences in the differentiating macronucleus display a distribution of PTMs which differs significantly from the PTM patterns of actively transcribed genes.

Conclusions
We show for the first time that a relocation of specific histone modifications takes place during activation of genes. In addition, we demonstrate that genes in a differentiating nucleus are characterised by a specific distribution and composition of PTMs. This allows us to propose a mechanistic model about the relevance of PTMs for gene activation, gene silencing and nuclear differentiation. Results described here will be relevant for eukaryotic cells in general.

The complete article is available as a provisional PDF

Complement-Derived Anaphylatoxin C3a Regulates In Vitro Differentiation and Migration of Neural Progenitor Cells

STEM CELLS
Volume 27 Issue 11;Pages 2824-2832
Published Online: 25;Sep;2009
Noriko Shinjyo ¹, Anders Ståhlberg ², Mike Dragunow ³, Milos Pekny ², Marcela Pekna ^{1 *¶1}Department of Medical Chemistry and Cell Biology, Institute of Biomedicine, Rehabilitation, Institute of Neuroscience and Physiology, Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden
²Center for Brain Repair and Rehabilitation, Department of Clinical Neuroscience and Rehabilitation, Institute of Neuroscience and Physiology, Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden
³Department of Pharmacology and The National Research Centre for Growth and Development, Faculty of Medical and Health Sciences, The University of Auckland, Auckland, New Zealand

Keywords: C3a, ERK1/2 phosphorylation, Neural progenitor cells, Neuronal differentiation, Migration, neuroprotection, neurogenesis.

New Discovery About Formation of New Brain Cells

ScienceDaily (Nov. 23, 2009) — The generation of new nerve cells in the brain is regulated by a peptide known as C3a, which directly affects the stem cells' maturation into nerve cells and is also important for the migration of new nerve cells through the brain tissue, reveals new research from the Sahlgrenska Academy published in the journal Stem Cells. (read more)

A Periplasmic Reducing System Protects Single Cysteine Residues from Oxidation

(Related to the previous post, the source paper of the previus news)

Science 20 November 2009, Vol. 326. no. 5956, pp. 1109 - 1111, DOI: 10.1126/science.1179557
Matthieu Depuydt,1 Stephen E. Leonard,2 Didier Vertommen,1 Katleen Denoncin,1 Pierre Morsomme,3 Khadija Wahni,4,5 Joris Messens,4,5 Kate S. Carroll,2 Jean-François Collet1,*

de Duve Institute, Université catholique de Louvain, B-1200 Brussels, Belgium.
2 Life Sciences Institute, University of Michigan, Ann Arbor, MI 48109–1048, USA.
3 Institut des Sciences de la Vie, Université catholique de Louvain, B-1348 Louvain-la-Neuve, Belgium.
4 Department of Molecular and Cellular Interactions, Vlaams Instituut voor Biotechnologie (VIB), Vrije Universiteit Brussel, B-1050 Brussels, Belgium.
5 Structural Biology Brussels, Vrije Universiteit Brussel, B-1050 Brussels, Belgium.

Keywords:  thiol group, cysteine,  disulfide bonds, oxidation,  sulfenic acids, sulfonic acids,  DsbG, DsbC,  thioredoxin-related proteins.

Saving The Single Cysteine: New Antioxidant System Found

Medical News Today, Article Date: 23 Nov 2009 - 1:00 PST
We've all read studies about the health benefits of having a life partner. The same thing is true at the molecular level, where amino acids known as cysteines are much more vulnerable to damage when single than when paired up with other cysteines.  read more....

Keywords: cysteines, new antioxidant system, DsbG, hyperoxidation, antioxidant therapies,

GUÍA PRÁCTICA DE NECESIDADES EDUCATIVAS ESPECIALES

Original book : At a glance: a practical guide to children's special needs

Autor: Viv EAST y Linda EVANS
Traductor: Pablo Manzano
ISBN: 978-84-7112-609-2
Fecha de la edición: 25/11/2009

Keywors: Necesidades Educativas Especiales, Ataxia de Friedreich, Plan Educativo Individualizado (PEI)

Búsqueda y análisis de fármacos en modelos de ataxia de Friedreich

Ros Cantera, Sheila,
Tesis doctoral del Departamento de Genética de la UV.
Editorial: Instituto de Biomedicina de Valencia, CSIC; Universitat de València, Fecha de publicación: 17-Sep-2009

Keywords: Ataxia de Friedreich, Frataxina, Riboflavina, deferiprona, deferoxamina, amrinona, crisina,


Trabajo completo / full text : http://digital.csic.es/bitstream/10261/17120/1/Tesis%20Sheila%20Ros.pdf

Residential Design For Persons With Neurological Disability

NeuroRehabilitation, Volume 25, Issue 3 (November 2009) Published by IOS Press

Content:

-Rehabilitation Design

-Evidence-based long term care design

-Enhancing quality of life through Universal Design

-Ten new and emerging trends in residential group living environments
 

-Enriching lighting design

-Residential design and outdoor area accessibility

-Smart homes for people with neurological disability: State of the art

-Residential design for real life rehabilitation

Epigenetic Silencing in Friedreich Ataxia Is Associated with Depletion of CTCF (CCCTC-Binding Factor) and Antisense Transcription

OPEN ACCESS

PLoS ONE 4(11): e7914. doi:10.1371/journal.pone.0007914

Irene De Biase^1#, Yogesh K. Chutake^1#, Paul M. Rindler¹, Sanjay I. Bidichandani^1,2*

1 Department of Biochemistry and Molecular Biology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, United States of America, 2 Department of Pediatrics, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, United States of America

Abstract

 

Background

Over 15 inherited diseases are caused by expansion of triplet-repeats. Friedreich ataxia (FRDA) patients are homozygous for an expanded GAA triplet-repeat sequence in intron 1 of the FXN gene. The expanded GAA triplet-repeat results in deficiency of FXN gene transcription, which is reversed via administration of histone deacetylase inhibitors indicating that transcriptional silencing is at least partially due to an epigenetic abnormality.

Methodology/Principal Findings

We found a severe depletion of the chromatin insulator protein CTCF (CCCTC-binding factor) in the 5′UTR of the FXN gene in FRDA, and coincident heterochromatin formation involving the +1 nucleosome via enrichment of H3K9me3 and recruitment of heterochromatin protein 1. We identified FAST-1 (FXN Antisense Transcript – 1), a novel antisense transcript that overlaps the CTCF binding site in the 5′UTR, which was expressed at higher levels in FRDA. The reciprocal relationship of deficient FXN transcript and higher levels of FAST-1 seen in FRDA was reproduced in normal cells via knockdown of CTCF.

Conclusions/Significance

CTCF depletion constitutes an epigenetic switch that results in increased antisense transcription, heterochromatin formation and transcriptional deficiency in FRDA. These findings provide a mechanistic basis for the transcriptional silencing of the FXN gene in FRDA, and broaden our understanding of disease pathogenesis in triplet-repeat diseases.

 Full text (pdf) http://www.plosone.org/article/fetchObjectAttachment.action;jsessionid=F09024199E18C3B55BF5F22C106E7255?uri=info%3Adoi%2F10.1371%2Fjournal.pone.0007914&representation=PDF

Excellent initiative to spread, and promote scientific research on rare diseases

Public Television of Catalunya, TV3, is doing important work through the "Fundació la Marató deTV3" ,this year focuses on rare diseases.

Yeast frataxin mutants display decreased superoxide dismutase activities crucial to promote protein oxidative damage

Free Radical Biology and Medicine, doi:10.1016/j.freeradbiomed.2009.11.010
Verónica Irazustaa, Elia Obisa, Armando Moreno-Cermeñoa, Elisa Cabiscola, Joaquim Ros, a, and Jordi Tamarita, Grup de Bioquímica de l’Estrés Oxidatiu, Departament de Ciències Mèdiques Bàsiques, Facultat de Medicina, Universitat de Lleida, Lleida, Spain.

Keywords: Iron-overload; protein carbonylation; metal-catalyzed oxidation; frataxin; magnesium-binding proteins, Mn-SOD, CuZn-SOD, superoxide dismutase; iron toxicity; Friedreich ataxia

Safety Study of Carbamylated Erythropoietin to Treat Patients With the Neurodegenerative Disorder Friedreich's Ataxia

This study is currently recruiting participants.

First Received: November 18, 2009   No Changes Posted

Sponsor:	H. Lundbeck A/S
Information provided by:	H. Lundbeck A/S
ClinicalTrials.gov Identifier:	NCT01016366

A Nanomedicine Transports a Peptide Caspase-3 Inhibitor across the Blood–Brain Barrier and Provides Neuroprotection

The Journal of Neuroscience, November 4, 2009, 29(44):13761-13769; doi:10.1523/JNEUROSCI.4246-09.2009

Hulya Karatas,1 Yesim Aktas,2 Yasemin Gursoy-Ozdemir,1 Ebru Bodur,3 Muge Yemisci,1 Secil Caban,2 Atay Vural,1 Onur Pinarbasli,2 Yilmaz Capan,2 Eduardo Fernandez-Megia,4 Ramon Novoa-Carballal,4 Ricardo Riguera,4 Karine Andrieux,5 Patrick Couvreur,5 and Turgay Dalkara1

1Department of Neurology, Faculty of Medicine and Institute of Neurological Sciences and Psychiatry, 2Department of Pharmaceutical Technology, Faculty of Pharmacy, and 3Department of Biochemistry, Faculty of Medicine, Hacettepe University, 06100 Ankara, Turkey, 4Departamento de Química Orgánica, Facultad de Química, and Unidad de Resonancia Magnética Nuclear de Biomoléculas Asociada al Consejo Superior de Investigaciones Científicas, Universidad de Santiago de Compostela, 15782 Santiago de Compostela, Spain, and 5Physico-Chimie, Pharmacotechnie, Biopharmacie, Faculté de Pharmacie, Université Paris Sud, UMR Centre National de la Recherche Scientifique 8612, 92296 Chatenay Malabry, France

Keywords:  cross the blood–brain barrier (BBB), nanocarrier system,chitosan nanospheres, caspase-3 inhibitor, Polyethylene glycol-coated nanospheres, intravenously injected,  neuroprotection, efficient penetration, brain delivery,  treatment of CNS disorders.

On Your Last Nerve: Researchers Advance Understanding of Stem Cells

ScienceDaily (Nov. 17, 2009) — Researchers from North Carolina State University have identified a gene that tells embryonic stem cells in the brain when to stop producing nerve cells called neurons. The research is a significant advance in understanding the development of the nervous system, which is essential to addressing conditions such as Parkinson's disease, Alzheimer's disease and other neurological disorders.

Read more ...

Acute Beta-adrenergic Stimulation does not Alter Mitochondrial Protein Synthesis or Markers of Mitochondrial Biogenesis in Adult Men.

Am J Physiol Regul Integr Comp Physiol. 2009 Nov 11

Robinson MM, Richards JC, Hickey MS, Moore DR, Phillips SM, Bell C, Miller BF.
Colorado State University.

Keywords: peroxisome proliferator-activated receptor gamma coactivator-1 alpha (PGC-1alpha), beta-adrenergic receptor (beta-AR) antagonist, skeletal muscle PGC-1alpha expression, mitochondrial biogenesis, whole body protein turnover (WBPT), myofibrillar protein synthesis (MyPS), skeletal muscle mitochondrial protein synthesis (MiPS), mitochondrial biogenic signalling, non-specific beta-AR agonist (isoproterenol (ISO),PGC-1alpha, TFAM, NRF-1, NRF-2, COX, NADHox.

Frataxin (FXN); histone deacetylase 3 (HDAC3)

SciBX: Science-Business eXchange 2, (2009) | doi:10.1038/scibx.2009.1637.

Kwywords: cell culture, HDAC3, Friedreich's ataxia, neurodegenerative, FXN gene.

Neuroprotection using gene therapy to induce vascular endothelial growth factor-A expression

Gene Therapy (2009) 16, 1292–1299; doi:10.1038/gt.2009.111;
S A Sakowski1, S B Heavener2,5, J S Lunn1, K Fung3, S S Oh1, S K Spratt4, N D Hogikyan2 and E L Feldman1

1Department of Neurology, University of Michigan Medical Center, Ann Arbor, MI, USA
2Department of Otolaryngology-Head and Neck Surgery, University of Michigan Medical Center, Ann Arbor, MI, USA
3Department of Otolaryngology, University of Western Ontario, London, Ontario, Canada
4Sangamo BioSciences, Inc., Richmond, CA, USA

Keywords:zinc-finger protein (ZFP) transcription factors, adenoviral vectors, Ad-32Ep65-Flag (Ad-p65), VEGF, increase in axon outgrowth, neuroprotective effects, laryngeal nerve (RLN)-crush injury, nerve regeneration, nerve injury, neurodegeneration.

Vascular niche factor PEDF modulates Notch-dependent stemness in the adult subependymal zone

Nature Neuroscience

Published online: 8 November 2009
doi:10.1038/nn.2437
Celia Andreu-Agulló, José Manuel Morante-Redolat, Ana C Delgado & Isabel Fariñas.
About stem cells, neurodegeneration, not specific Friedreich ataxia
Keywords: stem cell microenvironments, regulate self-renewal,  astroglia-like neural stem cells (NSCs), Notch transcriptional activity,  multipotency, Pigment epithelium–derived factor (PEDF), NF-B pathway, N-CoR.
 
Link To Full text: http://www.nature.com/neuro/journal/vaop/ncurrent/full/nn.2437.html

The specificity of neuroprotection by antioxidants

Journal of Biomedical Science 2009, 16:98doi:10.1186/1423-0127-16-98

Published: 5 November 2009

Yuanbin Liu and David R Schubert


Cellular Neurobiology Laboratory, The Salk Institute for Biological Studies,10010 N. Torrey Pines Road, La Jolla, California 92037-1099 USA


OPEN ACCESS

Abstract (provisional)


Background

Reactive oxygen species (ROS) play an important role in aging and age-related diseases such as Parkinson's disease and Alzheimer's disease. Much of the ROS production under conditions of toxic stress is from mitochondria, and multiple antioxidants prevent ROS accumulation. The aim of this study is to examine the specificity of the interaction between the antioxidants and ROS production in stressed cells.

Methods

Using fluorescent dyes for ROS detection and mitochondrial inhibitors of known specificities, we studied ROS production under three conditions where ROS are produced by mitochondria: oxidative glutamate toxicity, state IV respiration induced by oligomycin, and tumor necrosis factor-induced cell death.

Results

We demonstrated that there are at least four mitochondrial ROS-generating sites in cells, including the flavin mononucleotide (FMN) group of complex I and the three ubiquinone-binding sites in complexes I, II and III. ROS production from these sites is modulated in an insult-specific manner and the sites are differentially accessible to common antioxidants.

Conclusions

The inhibition of ROS accumulation by different antioxidants is specific to the site of ROS generation as well as the antioxidant. This information should be useful for devising new interventions to delay aging or treat ROS-related diseases.



Link to full text: http://www.jbiomedsci.com/content/pdf/1423-0127-16-98.pdf

Auditory Perception in Individuals with Friedreich's Ataxia

Audiol Neurotol 2010;15:229-240 (DOI: 10.1159/000255341)
Gary Rancea, Louise Corbenb, Elizabeth Barkera, Peter Carewa, Donella Chisaria, Meghan Rogersa, Richard Dowella, Saiful Jamaluddina, Rochelle Brysona, Martin B. Delatyckib

aDepartment of Otolaryngology, The University of Melbourne, and
bMurdoch Children's Research Institute, Parkville, Vic., Australia

Keywords: Friedreich's ataxia, Auditory perception, Temporal processing, Speech perception.

Auditory plasticity and speech motor learning

Sazzad M. Nasira and David J. Ostrya,
Department of Psychology, McGill University, Montreal, QC, Canada H3A 1B1; and  Haskins Laboratories, New Haven, CT 06511

Edited by Michael M. Merzenich, University of California, San Francisco, CA, and approved September 24, 2009 (received for review June 28, 2009)

OPEN ACCES
 
Abstract


Is plasticity in sensory and motor systems linked? Here, in the context of speech motor learning and perception, we test the idea sensory function is modified by motor learning and, in particular, that speech motor learning affects a speaker's auditory map. We assessed speech motor learning by using a robotic device that displaced the jaw and selectively altered somatosensory feedback during speech. We found that with practice speakers progressively corrected for the mechanical perturbation and after motor learning they also showed systematic changes in their perceptual classification of speech sounds. The perceptual shift was tied to motor learning. Individuals that displayed greater amounts of learning also showed greater perceptual change. Perceptual change was not observed in control subjects that produced the same movements, but in the absence of a force field, nor in subjects that experienced the force field but failed to adapt to the mechanical load. The perceptual effects observed here indicate the involvement of the somatosensory system in the neural processing of speech sounds and suggest that speech motor learning results in changes to auditory perceptual function.

link to full text: http://www.pnas.org/content/early/2009/10/30/0907032106.full.pdf

Researchers Unlock The 'Sound Of Learning' By Linking Sensory And Motor Systems

Medicalnewstoday, Article Date: 04 Nov 2009 - 0:00 PST

Learning to talk also changes the way speech sounds are heard, according to a new study published in Proceedings of the National Academy of Sciences by scientists at Haskins Laboratories, a Yale-affiliated research laboratory. The findings could have a major impact on improving speech disorders. (read more)

Frataxin interacts with Isu1 through a conserved tryptophan in its beta-sheet.

Hum Mol Genet. 2009,  Nov 2.

Leidgens S, De Smet S, Foury F.

Unité de Biochimie Physiologique, Institut des Sciences de la Vie, Université Catholique de Louvain, Croix du Sud 5-15, 1348 Louvain-la-Neuve, Belgium.

Keywords: Friedreich's ataxia, frataxin, iron-sulfur (Fe/S) clusters, Yfh1, Isu1, beta-sheet platform, Q129A, I130A, W131A(F), R141A, low aconitase activity, Gln-129, Trp-131, Arg-141, aromatic side chain.

Iron Deficiency is the Most Common Deficiency Among People

November 02, 2009
http://www.associatedcontent.com/

Keyword: Dr. Timothy Stemmler, Wayne State University, University of Michigan-Dearborn, iron deficiency, Anemia, Parkinson's, Fredreich's Ataxia,  frataxin.

Lundbeck starts clinical phase IIa with Lu AA24493 (cEPO) in Friedreich's ataxia in a study also assessing efficacy via biomarkers

PR-inside.com,
London, November , 02, 2009
 
Keywords: H. Lundbeck A/S, phase IIa clinical studies, project Lu AA24493, safety  and
tolerability,  Friedreich's ataxia,  carbamoylated form of human erythropoietin (EPO), loss of haematopoietic effects,  neuronal damage,

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Pharmacy Europe,
Latest News, Monday 2nd November 2009

Lundbeck starts clinical phase IIa with Lu AA24493 (cEPO)

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Reuters.com
Mon Nov 2, 2009 5:10am EST

Lundbeck says expands stroke drug candidate trials