Epigenetic Enhancement of BDNF Signaling Rescues Synaptic Plasticity in Aging

The Journal of Neuroscience, 7 December 2011, 31(49): 17800-17810; doi: 10.1523/​JNEUROSCI.3878-11.2011

Yan Zeng, Miao Tan, Jun Kohyama, Marissa Sneddon, Joseph B. Watson, Yi E. Sun, and
Cui-Wei Xie
Department of Psychiatry and Biobehavioral Sciences, Semel Institute for Neuroscience and Human Behavior, University of California, Los Angeles, California 90024

Keywords: Aging-related cognitive declines, synaptic plasticity, dendritic spines, reduced histone acetylation, brain-derived neurotrophic factor (Bdnf) gene, H3 and H4 acetylation, HDAC inhibition, 7,8-dihydroxyflavone, trkB, epigenetic or pharmacological.

Substantia nigra hypoechogenicity in friedreich ataxia

Movement Disorders, Article first published online: 9 DEC 2011 | DOI: 10.1002/mds.23989

Heike Stockner MD, Martin Sojer MD, Sascha Hering MD, Wolfgang Nachbauer MD, Klaus Seppi MD, Christoph Schmidauer MD, Werner Poewe MD, Sylvia

Mutations in the dimer interface of dihydrolipoamide dehydrogenase promote site-specific oxidative damages in yeast and human cells

J. Biol. Chem. jbc.M111.274415. First Published on September 19, 2011, doi:10.1074/jbc.M111.274415

Rachael A. Vaubel, Pierre Rustin, and Grazia Isaya


DLD dimer interface mutations may accelerate frataxin turnover via an unknown mechanism

The role of DLD proteolytic activity remains unclear, and will require a better understanding of conditions that accelerate FXN turnover and the identification of additional natural substrates cleaved by DLD.

Structure-function study of cellular iron chemistry

OhioLINK ETD Center, Document number: osu1245414801

Huang, Jia

Keywords: iron-sulfur clusters, frataxin, ferrous iron, scaffold protein human ISU, cytosolic aconitase, IRP1, HscB, ITC.

The yeast metacaspase is implicated in oxidative stress response in frataxin-deficient cells

FEBS Letters - 07 December 2011 (10.1016/j.febslet.2011.12.002)

Sophie Lefevre, Dominika Sliwa, Françoise Auchère, Caroline Brossas, Christoph Ruckenstuhl, Nicole Boggetto, Emmanuel Lesuisse, Frank Madeo, Jean-Michel Camadro, Renata Santos

Keywords: Friedreich ataxia, mitochondrial frataxin, iron-sulfur cluster, heme biosynthesis, antioxidant therapies, hydrogen peroxide, metacaspase.

Posttranslational Modification of Cysteine in Redox Signaling and Oxidative Stress – Focus on S-Glutathionylation

Antioxidants & Redox Signaling. null, Vol. 0, No. ja

Prof. John J. Mieyal, Case Western Reserve University, School of Medicine, Pharmacology, Cleveland, Ohio, United States.
Boon Chock, NHLBI, National Institutes of Health, Laboratory of Biochemistry, Bethesda, Maryland, United States.

Keywords: Reactive oxygen species (ROS), reactive nitrogen species (RNS), cellular signaling pathways, peroxynitrite, cysteine residues, reversible protein-S-glutathionylation, mitochondria, cardiovascular system, neurodegenerative diseases.

DiseaseMeth: a human disease methylation database

Nucl. Acids Res. (2011) doi: 10.1093/nar/gkr1169

Jie Lv, Hongbo Liu, Jianzhong Su, Xueting Wu, Hui Liu, Boyan Li, Xue Xiao, Fang Wang, Qiong Wu, and Yan Zhang

OPEN ACCESS

Keywords: DNA methylation, epigenetic modification, genomic regulation, disease biomarkers, DNA methylation repository, DiseaseMeth, human disease methylation database, gene–disease relationship.

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Normal left ventricular ejection fraction and mass but subclinical myocardial dysfunction in patients with Friedreich's ataxia.

Eur J Echocardiogr. 2011 Nov 28.

Dedobbeleer C, Rai M, Donal E, Pandolfo M, Unger P.
Department of Cardiology, Erasme Hospital, Université Libre de Bruxelles, 808 Rte de Lennik, Brussels 1070, Belgium.

Keywords: Myocardial involvement, Friedreich's ataxia (FRDA), iron deposits, diffuse fibrosis, focal necrosis.

There is evidence of morphological and functional abnormalities in FRDA patients with normal LVEF and mass.

Specialized Function of Yeast Isa1 and Isa2 Proteins in the Maturation of Mitochondrial [4Fe-4S] Proteins

J. Biol. Chem. 2011 286: 41205-41216. First Published on October 10, 2011, doi:10.1074/jbc.M111.296152

Ulrich Mühlenhoff,
Nadine Richter,
Ophry Pines,
Antonio J. Pierik,
and Roland Lill


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FXN methylation predicts expression and clinical outcome in friedreich ataxia

Annals of Neurology, Accepted manuscript online: 25 NOV 2011 08:37AM EST | DOI: 10.1002/ana.22671

Marguerite V. Evans-Galea, Nissa Carrodus, Simone M. Rowley, Louise A. Corben, Geneieve Tai, Richard Saffery, John C. Galati, Nicholas C. Wong, Jeffrey M. Craig, David R. Lynch, Sean Regner, Alicia F. D. Brocht, Susan L. Perlman, Khalaf O. Bushara, Christopher M. Gomez, George R. Wilmot, Lingli Li, Elizabeth Varley, Martin B. Delatycki and Joseph P. Sarsero

Keywords: Friedreich ataxia (FRDA), expanded GAA repeat, frataxin, epigenetic, clinical parameters, DNA methylation, age of onset, Friedreich Ataxia Rating Scale, disease severity, biomarker.