Cerebellar damage impairs the self-rating of regret feeling in a gambling task

Cerebellar damage impairs the self-rating of regret feeling in a gambling task. Clausi S, Coricelli G, Pisotta I, Pavone EF, Lauriola M, Molinari M and Leggio M (2015); Front. Behav. Neurosci. 9:113. doi: 10.3389/fnbeh.2015.00113

Seminar: Dr. Javier Díaz-Nido, Friedreich's Ataxia as model for neurodegenerative diseases: From molecular biology to the quest for therapies

Seminar: Dr. Javier Díaz-Nido, Friedreich's Ataxia as model for neurodegenerative diseases: From molecular biology to the quest for therapies. 2015-06-0414:00 Petrén lecture hall, Nobels väg 12b (Solna).Campus Solna

Frataxin accelerates [2Fe-2S] cluster formation on the human Fe-S assembly complex

Frataxin accelerates [2Fe-2S] cluster formation on the human Fe-S assembly complex. Nicholas G. Fox , Deepika Das , Mrinmoy Chakrabarti , Paul Alan Lindahl , and David P. Barondeau, Biochemistry, Just Accepted Manuscript DOI: 10.1021/bi5014497 Publication Date (Web): May 27, 2015

Nuclear-mitochondrial proteins: too much to process

Nuclear-mitochondrial proteins: too much to process. Rita Horvath , Patrick F. Chinnery, Brain First published online: 26 May 2015, DOI: http://dx.doi.org/10.1093/brain/awv072 1451-1453



Trinucleotide repeat expansions and point mutations in FXN, which codes for frataxin, cause the most common form of autosomal recessive ataxia—Friedreich’s ataxia—providing a link between PMPCA and the ataxia affecting the 17 patients described by Jobling et al.

Inhibition of the tyrosine kinase Src might be a new strategy for treating Friedreich ataxia

Inhibition of the tyrosine kinase Src might be a new strategy for treating Friedreich ataxia. Nature Reviews Neurology (2015) Research Highlight-In brief, doi:10.1038/nrneurol.2015.89 Published online 26 May 2015

Original article Cherubini, F. et al. Src inhibitors modulate frataxin protein levels. Hum. Mol. Genet. doi:10.1093/hmg/ddv162

Turning Escherichia coli into a Frataxin-Dependent Organism

Turning Escherichia coli into a Frataxin-Dependent Organism. Roche B, Agrebi R, Huguenot A, Ollagnier de Choudens S, Barras F, Py B (2015), PLoS Genet 11(5): e1005134. doi:10.1371/journal.pgen.1005134

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Trading Places—Switching Frataxin Function by a Single Amino Acid Substitution within the [Fe-S] Cluster Assembly Scaffold

Trading Places—Switching Frataxin Function by a Single Amino Acid Substitution within the [Fe-S] Cluster Assembly Scaffold. Dean DR, Dos Santos PC (2015) PLoS Genet 11(5): e1005192. doi:10.1371/journal.pgen.1005192

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Turning Saccharomyces cerevisiae into a Frataxin-Independent Organism

Turning Saccharomyces cerevisiae into a Frataxin-Independent Organism. Yoon H, Knight SAB, Pandey A, Pain J, Turkarslan S, Pain D, Andrew Dancis. (2015) PLoS Genet 11(5): e1005135. doi:10.1371/journal.pgen.1005135

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Use of MAPK pathway inhibitors for the treatment of Friedreich Ataxia

Use of MAPK pathway inhibitors for the treatment of Friedreich Ataxia . University of Pennsylvania, posted on 05/19/2015

Brief Description: A novel treatment for rare disease Friedreich ataxia using p38 or MK2 kinase inhibitors

A First in Human Study of RT001 in Patients With Friedreich's Ataxia

A First in Human Study of RT001 in Patients With Friedreich's Ataxia.ClinicalTrials.gov Identifier: NCT02445794, Sponsor: Retrotope, Inc.

Study Type: Interventional
Study Design: Allocation: Randomized
Endpoint Classification: Safety Study
Intervention Model: Parallel Assignment
Masking: Double Blind (Subject, Caregiver, Investigator, Outcomes Assessor)
Primary Purpose: Treatment

Estimated Enrollment: 18
Study Start Date: July 2015
Estimated Study Completion Date: February 2016
Estimated Primary Completion Date: January 2016 (Final data collection date for primary outcome measure)