The optic nerve: A “mito-window” on mitochondrial neurodegeneration

The optic nerve: A “mito-window” on mitochondrial neurodegeneration. Alessandra Maresca, Chiara la Morgia, Leonardo Caporali, Maria Lucia Valentino, Valerio Carelli; Molecular and Cellular Neuroscience, Available online 14 August 2012, ISSN 1044-7431, 10.1016/j.mcn.2012.08.004. (http://www.sciencedirect.com/science/article/pii/S1044743112001315?v=s5)

 Keywords: Mitochondrial disease, Optic atrophy, Mitochondrial functions, LHON, DOA, Friedreich's Ataxia, Retinal ganglion cells

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Job vacancies at Laboratory of Annalisa Pastore, MRC National Institute for Medical Research, London

Career Development Fellowship, Structural characterization of the iron sulfur cluster biogenesis machinery, Division of Molecular Structure, Laboratory of Annalisa Pastore

Heterotrifunctional chemical crosslinking mass spectrometry confirms physical interaction between human frataxin and ISU

Heterotrifunctional chemical crosslinking mass spectrometry confirms physical interaction between human frataxin and ISU. Watson HM, Gentry LE, Asuru AP, Wang Y, Marcus S, Busenlehner LS.; Biochemistry. 2012 Aug 16. [Epub ahead of print]

Keywords: Friedreich's ataxia, frataxin, iron chaperone, iron-sulfur clusters, new protein partners and pathways.

Management and therapy for cardiomyopathy in Friedreich’s ataxia

Management and therapy for cardiomyopathy in Friedreich’s ataxia.David R Lynch, Sean R Regner, Kimberly A Schadt, Lisa S Friedman, Kimberly Y Lin and Martin G St John Sutton. Expert Review of Cardiovascular Therapy, June 2012, Vol. 10, No. 6 , Pages 767-777 (doi: 10.1586/erc.12.57) Keywords: Friedreich’s ataxia, cardiomyopathy, congestive heart failure, ventricular arrhythmias, cardio–embolic stroke.

Iron-Sulfur Cluster Synthesis, Iron Homeostasis and Oxidative Stress in Friedreich Ataxia

Iron-Sulfur Cluster Synthesis, Iron Homeostasis and Oxidative Stress in Friedreich Ataxia; Rachael A. Vaubel, Grazia Isaya; Molecular and Cellular Neuroscience, Available online 10 August 2012, http://dx.doi.org/10.1016/j.mcn.2012.08.003

Keywords: frataxin, mitochondria, heme, oxidative stress, anti-oxidants, iron-chelators, ISC, iron-sulfur clusters, FRDA, Friedreich ataxia, NFS1/Nfs1, human/yeast cysteine desulfurase, ISD11/Isd11, human/yeast NFS1-/Nfs1-binding protein, ISCU/Isu1, human/yeast scaffold protein, MPP, mitochondrial processing peptidase.

The Essential Iron-Sulfur Protein Rli1 is an Important Target Accounting for Inhibition of Cell Growth by Reactive Oxygen Species

The Essential Iron-Sulfur Protein Rli1 is an Important Target Accounting for Inhibition of Cell Growth by Reactive Oxygen Species.Alhebshi A, Sideri TC, Holland SL, Avery SV.; Mol Biol Cell. 2012 Aug 1. Keywords: Oxidative stress, reactive oxygen species (ROS), molecular target(s), Rli1p (ABCE1), ROS-labile cofactors (Fe-S clusters).

Role of frataxin in erythropoietin- and carbamoylated erythropoietin- mediated neuroprotective effects in the mouse retina after acute retinal ischemia/reperfusion in vivo

Role of frataxin in erythropoietin- and carbamoylated erythropoietin- mediated neuroprotective effects in the mouse retina after acute retinal ischemia/reperfusion in vivo. Rowena Schultz, Otto W Witte, Christian Schmeer F1000 Posters 2012, 3: 889 (poster); Presented at 7th International Symposium on Neuroprotection and Neurorepair 2012, 2 - 5 May 2012, PIII -40

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Quantitative Analysis to Guide Orphan Drug Development

Quantitative Analysis to Guide Orphan Drug Development.L J Lesko. Clinical Pharmacology & Therapeutics (2012); 92 2, 258–261. doi:10.1038/clpt.2012.80

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Neuroprotection with non-feminizing estrogen analogues: An overlooked possible therapeutic strategy

Neuroprotection with non-feminizing estrogen analogues: An overlooked possible therapeutic strategy.James W. Simpkins, Timothy E. Richardson, Kun Don Yi, Evelyn Perez, Douglas F. Covey; Hormones and Behavior, Available online 3 April 2012. http://dx.doi.org/10.1016/j.yhbeh.2012.03.013. This article is part of a Special Issue entitled Hormones & Neurotrauma.

Keywords: estrogens, estrogen receptors (ERs), neuroprotective activity, non-ER mechanisms, synthetic non-feminizing estrogens, phenolic, Friedreich's Ataxia, cell model, oxidative stress, Hormone therapy.

Unintended effects of orphan product designation for rare neurological diseases

Unintended effects of orphan product designation for rare neurological diseases, Sinéad M Murphy, Araya Puwanant and Robert C. Griggs, the CINCH IHC Consortia of the Rare Disease Clinical Research Network Unintended effects of orphan product designation for rare neurological diseases. Annals of Neurology, Accepted manuscript online: 22 JUN 2012 03:53AM EST | DOI: 10.1002/ana.23672.