RNA-INDUCED TRANSCRIPTIONAL GENE SILENCING IN FRIEDREICH ATAXIA

RNA-INDUCED TRANSCRIPTIONAL GENE SILENCING IN FRIEDREICH ATAXIA. PROJECT: NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE

Project Number: 5R01NS072418-04
Project Leader: BIDICHANDANI, SANJAY I
Awardee Organization: UNIVERSITY OF OKLAHOMA HLTH SCIENCES CTR

Turn up the power - pharmacological activation of mitochondrial biogenesis in mouse models

Turn up the power - pharmacological activation of mitochondrial biogenesis in mouse models. J C Komen and D R Thorburn; British Journal of Pharmacology, Accepted manuscript online: 17 SEP 2013 11:12PM EST | DOI: 10.1111/bph.12413

Keywords: mitochondria; oxidative phosphorylation; PGC-1α; mitochondrial biogenesis; bezafibrate; resveratrol; AICAR; mouse models; mitochondrial disease

Exercise Induces Hippocampal BDNF through a PGC-1α/FNDC5 Pathway

Exercise Induces Hippocampal BDNF through a PGC-1α/FNDC5 Pathway. Christiane D. Wrann, James P. White, John Salogiannnis, Dina Laznik-Bogoslavski, Jun Wu, Di Ma, Jiandie D. Lin, Michael E. Greenberg, Bruce M. Spiegelman; Cell Metabolism, Available online 10 October 2013. http://dx.doi.org/10.1016/j.cmet.2013.09.008

This findings link endurance exercise and the important metabolic mediators, PGC-1α and FNDC5, with BDNF expression in the brain.

Methylene blue rescues heart defects in a Drosophila model of Friedreich's ataxia

Methylene blue rescues heart defects in a Drosophila model of Friedreich's ataxia. Hervé Tricoire, Amandine Palandri, Arthur Bourdais, Jean-Michel Camadro and Véronique Monnier. Hum. Mol. Genet. (2013) doi: 10.1093/hmg/ddt493.

KEYWORDS: Friedreich's Ataxia (FRDA), Drosophila, neurons, glial cells, methylene blue (MB), alternative electron carrier.

A Phenotypic Screen Identifies Cyclic Peptide Compounds that Induce Repeat Expansion in Yeast

A Phenotypic Screen Identifies Cyclic Peptide Compounds that Induce Repeat Expansion in Yeast.Nicholas G. Economos, An honors thesis for the Department of Biology, Tufts University, 2013.

FULL TEXT

The iron-sulfur cluster assembly machineries in plants: current knowledge and open questions

The iron-sulfur cluster assembly machineries in plants: current knowledge and open questions. Couturier J, Touraine B, Briat J-F, Gaymard F and Rouhier N, Front. Plant Sci. 4:259. doi: 10.3389/fpls.2013.00259

Review ARTICLE

Keywords: iron-sulfur, assembly machineries, iron donor, repair, scaffold proteins, carrier Proteins, frataxin

Comparison of somatosensory evoked potentials between adolescent idiopathic scoliosis and congenital scoliosis without neural axis abnormalities

Comparison of somatosensory evoked potentials between adolescent idiopathic scoliosis and congenital scoliosis without neural axis abnormalities . Zhijun Chen, Yong Qiu, Weiwei Ma, Bangping Qian, Zezhang Zhu; The Spine Journal, Available online 5 October 2013. DOI: http://dx.doi.org/10.1016/j.spinee.2013.07.465.

Much evidence in recent years has shown neurologic dysfunction to be regarded as an ultimate cause for adolescent idiopathic scoliosis (AIS) and. In growing children, diseases that alter posterior column function (eg, Chiari I malformation, syringomyelia, and Friedreich ataxia) are consistently associated with an idiopathic (rather than a neuromuscular) curve pattern.

Flexible Cavovarus Foot in Children and Adolescents

Flexible Cavovarus Foot in Children and Adolescents. Kelly L. VanderHave, Robert N. Hensinger, Brandon W. King; Foot and Ankle Clinics, Available online 5 October 2013. DOI: http://dx.doi.org/10.1016/j.fcl.2013.08.006

Keywords: Cavovarus feet; Plantar fascia; Calcaneonavicular ligament; Calcaneocuboid ligament; neurologic diseases; Charcot-Marie-Tooth; Friedreich ataxia

Metabolic remodeling in frataxin-deficient yeast is mediated by Cth2 and Adr1

Metabolic remodeling in frataxin-deficient yeast is mediated by Cth2 and Adr1. Armando Moreno-Cermeño, David Alsina, Elisa Cabiscol, Jordi Tamarit, Joaquim Ros. Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, Available online 5 October 2013. DOI: http://dx.doi.org/10.1016/j.bbamcr.2013.09.019

Keywords: Friedreich ataxia; yeast frataxin; iron; oxidative stress

EFACTS’ (European Friedreich’s Ataxia Consortium for Translational Studies) Annual Newsletter 2013.

EFACTS’ (European Friedreich’s Ataxia Consortium for Translational Studies) Annual Newsletter 2013.

This third issue of EFACTS NEWS presents an update on the status of the EFACTS patient registry as baseline assessments are completed for a core sample of patients. Progress in the various research areas has been made over the past year. Patients are particularly interested in treatment options and may be glad to read that this newsletter contains information on progress in a clinical trial with a potential therapeutic candidate. There have been a number of publications on findings derived from EFACTS projects, which are also presented in this newsletter.