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