Monday, July 22, 2013

Abnormal body iron distribution and erythropoiesis in a novel mouse model with inducible gain of iron regulatory protein (IRP)-1 function

Abnormal body iron distribution and erythropoiesis in a novel mouse model with inducible gain of iron regulatory protein (IRP)-1 function. D. Casarrubea, L. Viatte, T. Hallas, A. Vasanthakumar, R. S. Eisenstein, K. Schümann, M. W. Hentze and B. Galy. J Mol Med (Berl). 2013 July; 91(7): 871–881. Published online 2013 March 1. doi: 10.1007/s00109-013-1008-2

It opens novel avenues to study diseases associated with abnormally high IRP1 activity, such as Parkinson’s disease or Friedreich’s ataxia.

Friday, July 19, 2013

Insights into the role of oxidative stress in the pathology of Friedreich Ataxia using peroxidation resistant polyunsaturated fatty acids

Insights into the role of oxidative stress in the pathology of Friedreich Ataxia using peroxidation resistant polyunsaturated fatty acids. M.Grazia Cotticelli, Andrew M. Crabbe, Robert B. Wilson, Mikhail S. Shchepinov; Redox Biology, Available online 19 July 2013. DOI: http://dx.doi.org/10.1016/j.redox.2013.06.004

Open Access Article

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Wednesday, July 17, 2013

Clinical features of Friedreich's ataxia: classical and atypical phenotypes

Clinical features of Friedreich's ataxia: classical and atypical phenotypes. Michael H. Parkinson, Sylvia Boesch, Wolfgang Nachbauer, Caterina Mariotti, Paola Giunti. J. Neurochem.(2013) 126 (Suppl. 1), 103–117. DOI: 10.1111/jnc.12317

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Increasing frataxin gene expression with histone deacetylase inhibitors as a therapeutic approach for Friedreich's ataxia

Increasing frataxin gene expression with histone deacetylase inhibitors as a therapeutic approach for Friedreich's ataxia. Joel M. Gottesfeld, James R. Rusche and Massimo Pandolfo. J. Neurochem.(2013) 126 (Suppl. 1), 147–154. DOI: 10.1111/jnc.12302

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Cardiomyopathy of Friedreich Ataxia

Cardiomyopathy of Friedreich Ataxia Frank Weidemann, Stefan Störk, Dan Liu, Kai Hu, Sebastian Herrmann, Georg Ertl and Markus Niemann. J. Neurochem.(2013) 126 (Suppl. 1), 88–93. DOI: 10.1111/jnc.12217

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Erythropoietin in Friedreich ataxia

Erythropoietin in Friedreich ataxia. Caterina Mariotti, Wolfgang Nachbauer, Marta Panzeri, Werner Poewe, Franco Taroni and Sylvia Boesch. J. Neurochem.(2013) 126 (Suppl. 1), 80–87. DOI: 10.1111/jnc.12301

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Animal and cellular models of Friedreich ataxia

Animal and cellular models of Friedreich ataxia. Morgane Perdomini, Aurore Hick, Hélène Puccio and Mark A. Pook. J. Neurochem.(2013) 126 (Suppl. 1), 65–79. DOI: 10.1111/jnc.12219

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Frataxin: a protein in search for a function

Frataxin: a protein in search for a function. Annalisa Pastore and Helene Puccio; J. Neurochem.(2013) 126 (Suppl. 1), 43–52. DOI: 10.1111/jnc.12220

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Gene regulation and epigenetics in Friedreich's ataxia

Gene regulation and epigenetics in Friedreich's ataxia. Cihangir Yandim, Theona Natisvili and Richard Festenstein. J. Neurochem.(2013) 126 (Suppl. 1), 21–42. DOI: 10.1111/jnc.12254

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Prevalence gradients of Friedreich's Ataxia and R1b haplotype in Europe co-localize, suggesting a common Palaeolithic origin in the Franco-Cantabrian ice age refuge

Prevalence gradients of Friedreich's Ataxia and R1b haplotype in Europe co-localize, suggesting a common Palaeolithic origin in the Franco-Cantabrian ice age refuge. Pierre Vankan; J. Neurochem.(2013) 126 (Suppl. 1), 11–20. DOI: 10.1111/jnc.12215

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