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