Médias tsiganes en France et en Hongrie : re-présentation de soi dans l’espace public

Cette contribution entend dresser un portrait des médias créés par et pour des Tsiganes en France et en Hongrie, via les supports traditionnels et sur Internet. L’analyse porte sur la représentation de soi dans l’espace public entreprise par des acteurs et actrices médiatiques œuvrant à renverser le déni du droit à s’auto-définir dont les Tsiganes font généralement l’objet. L’enjeu de ces présentations alternatives des groupes s’inscrit tant dans la conquête d’une dignité que de l’acquisition de droits ou de meilleures conditions de vie. Rares sont les recherches dans ces deux pays qui étudient les médias des minorités comme de véritables outils de lutte face aux rapports de domination.L’article analyse les conditions de l’émergence de cette parole collective dans l’espace public, en s’arrêtant sur les parcours biographiques des artisans de la nouvelle génération de ces médias, replacés dans leurs contextes nationaux et transnational. Il étudie également le rôle militant de ces productions dans leur contenu revendicatif et dans les diverses manières dont elles sont utilisées.This article aims to study the media created by and for Gypsies in France and Hungary, in traditional forms or on the Internet. It studies media actor’s self-re-presentation in public space. The challenges of these alternative representations are diverse: reversing the denial of self-definition, regaining some dignity, acquiring rights or better living conditions. In both countries, very little research has studied minority media as a tool against relations of domination.The author analyses the conditions of emergence of a collective voice in public space. She retraces the biographies of the people involved in these new media, in their national and transnational context. She also studies the militant use of these media through their political content and in the various ways that they are used.La presente contribución trata de mostrar un panorama de los medios creados por y para gitanos en Francia y en Hungría, tanto en los soportes tradiciones como en Internet. El análisis se centra en la re-presentación llevada a cabo en el espacio público por actores mediáticos que trabajan por hacer realidad el derecho de los gitanos a auto-definirse, un derecho que generalmente ha sido negado a esta población. El desafío de estas presentaciones alternativas de grupos se inscribe tanto en la conquista de una dignidad como en la adquisición de derechos y de mejores condiciones de vida. Pocas son las investigaciones en estos dos países que estudian los medios de minorías como verdaderas herramientas de lucha frente a las relaciones de dominación.Al artículo analiza las condiciones de emergencia de esta palabra colectiva en el espacio público, deteniéndose en las trayectorias biográficas de los artífices de la nueva generación de estos medios y situándolas en sus contextos nacional y transnacional. El artículo estudia además el papel militante de estas producciones no sólo en su contenido reivindicativo, sino también en las diversas formas en que se utilizan

Des médias par et pour les minoritaires ? (Re)production du genre et imbrication des rapports de domination

Ce numéro est issu du colloque international organisé en mars 2010 à la MSHS de Poitiers par l'équipe Minoritymedia sur « les médias des minorités ethniques, entre hégémonie et résistances ? ». Il reprend particulièrement les textes de la session consacrée au genre et à l'articulation des rapports de domination. Notre réflexion est ancrée dans une tradition intellectuelle qui analyse les pratiques culturelles au cœur même des systèmes hégémoniques. Nous nous appuyons sur la notion d'hégémonie..

Dyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia.

Inherited deficiency in the mitochondrial protein frataxin (FXN) causes the rare disease Friedreich's ataxia (FA), for which there is no successful treatment. We identified a redox deficiency in FA cells and used this to model the disease. We screened a 1600-compound library to identify existing drugs, which could be of therapeutic benefit. We identified the topical anesthetic dyclonine as protective. Dyclonine increased FXN transcript and FXN protein dose-dependently in FA cells and brains of animal models. Dyclonine also rescued FXN-dependent enzyme deficiencies in the iron-sulfur enzymes, aconitase and succinate dehydrogenase. Dyclonine induces the Nrf2 [nuclear factor (erythroid-derived 2)-like 2] transcription factor, which we show binds an upstream response element in the FXN locus. Additionally, dyclonine also inhibited the activity of histone methyltransferase G9a, known to methylate histone H3K9 to silence FA chromatin. Chronic dosing in a FA mouse model prevented a performance decline in balance beam studies. A human clinical proof-of-concept study was completed in eight FA patients dosed twice daily using a 1% dyclonine rinse for 1 week. Six of the eight patients showed an increase in buccal cell FXN levels, and fold induction was significantly correlated with disease severity. Dyclonine represents a novel therapeutic strategy that can potentially be repurposed for the treatment of FA

Characterising brown dwarf companions with IRDIS long-slit spectroscopy: HD 1160 B and HD 19467 B

The determination of the fundamental properties (mass, separation, age, gravity and atmospheric properties) of brown dwarf companions allows us to infer crucial informations on their formation and evolution mechanisms. Spectroscopy of substellar companions is available to date only for a limited number of objects (and mostly at very low resolution, R<50) because of technical limitations, i.e., contrast and angular resolution. We present medium resolution (R=350), coronagraphic long-slit spectroscopic observations with SPHERE of two substellar companions, HD 1160 B and HD 19467 B. We found that HD 1160 B has a peculiar spectrum that cannot be fitted by spectra in current spectral libraries. A good fit is possible only considering separately the Y+J and the H spectral band. The spectral type is between M5 and M7. We also estimated a T_eff of 2800-2900 K and a log(g) of 3.5-4.0 dex. The low surface gravity seems to favour young age (10-20 Myr) and low mass (~20 M Jup ) for this object. HD 19467 B is instead a fully evolved object with a T_eff of ~1000 K and log g of ~5.0 dex. Its spectral type is T6+/-1.Comment: 14 pages, 14 Figures. Accepted for publication on MNRA

Targeting lipid peroxidation and mitochondrial imbalance in Friedreich's ataxia

Friedreich's ataxia (FRDA) is an autosomal recessive disorder, caused by reduced levels of the protein frataxin. This protein is located in the mitochondria, where it functions in the biogenesis of iron-sulphur clusters (ISCs), which are important for the function of the mitochondrial respiratory chain complexes. Moreover, disruption in iron biogenesis may lead to oxidative stress. Oxidative stress can be the cause and/or the consequence of mitochondrial energy imbalance, leading to cell death. Fibroblasts from two FRDA mouse models, YG8R and KIKO, were used to analyse two different categories of protective compounds: deuterised poly-unsaturated fatty acids (dPUFAs) and Nrf2-inducers. The former have been shown to protect the cell from damage induced by lipid peroxidation and the latter trigger the well-known Nrf2 antioxidant pathway. Our results show that the sensitivity to oxidative stress of YG8R and KIKO mouse fibroblasts, resulting in cell death and lipid peroxidation, can be prevented by d4-PUFA and Nrf2-inducers (SFN and TBE-31). The mitochondrial membrane potential (ΔΨm) of YG8R and KIKO fibroblasts revealed a difference in their mitochondrial pathophysiology, which may be due to the different genetic basis of the two models. This suggests that variable levels of reduced frataxin may act differently on mitochondrial pathophysiology and that these two cell models could be useful in recapitulating the observed differences in the FRDA phenotype. This may reflect a different modulatory effect towards cell death that will need to be investigated further.RA is supported by FARA and GoFar, A&C Simeone Foundation.PG has received funding from FARA and GoFar, A&C Simeone Foundation and the European Union Seventh Framework Programme(FP7/2007-2013) under grant agreement number 242193/EFACTS.PG is supported by the National Institute for Health Research, University College London Hospitals, Biomedical Research Centre. EU and IR were supported by MSc scholarships

The CAG repeat at the Huntington disease gene in the Portuguese population : insights into its dynamics and to the origin of the mutation

Huntington disease (HD) is caused by an expansion of a CAG repeat. This repeat is a dynamic mutation that tends to undergo intergenerational instability. We report the analysis of the CAG repeat in a large population sample (2,000 chromosomes) covering all regions of Portugal, and a haplotype study of (CAG)n and (CCG)n repeats in 140 HD Portuguese families. Intermediate class 2 alleles represented 3.0% of the population; and two expanded alleles (36 and 40 repeats, 0.11%) were found. There was no evidence for geographical clustering of the intermediate or expanded alleles. The Portuguese families showed three different HD founder haplotypes associated with 7-, 9- or 10-CCG repeats, suggesting the possibility of different origins for theHDmutation among this population. The haplotype carrying the 7-CCG repeat was the most frequent, both in normal and in expanded alleles. In general, we propose that three mechanisms, occurring at different times,may lead to the evolution from normal CAGs to full expansion: first, a mutation bias towards larger alleles; then, a stepwise process that could explain the CAGdistributions observed in themore recent haplotypes; and, finally, a pool of intermediate (class 2) alleles more prone to give rise to expanded HD alleles.Fundação para a Ciência e a Tecnologia (FCT) - SFRH/BD/9759/ 2003.Instituto de Genética Médica Jacinto Magalhães

A Combined Nucleic Acid and Protein Analysis in Friedreich Ataxia: Implications for Diagnosis, Pathogenesis and Clinical Trial Design

BACKGROUND: Friedreich's ataxia (FRDA) is the most common hereditary ataxia among caucasians. The molecular defect in FRDA is the trinucleotide GAA expansion in the first intron of the FXN gene, which encodes frataxin. No studies have yet reported frataxin protein and mRNA levels in a large cohort of FRDA patients, carriers and controls. METHODOLOGY/PRINCIPAL FINDINGS: We enrolled 24 patients with classic FRDA phenotype (cFA), 6 late onset FRDA (LOFA), all homozygous for GAA expansion, 5 pFA cases who harbored the GAA expansion in compound heterozygosis with FXN point mutations (namely, p.I154F, c.482+3delA, p.R165P), 33 healthy expansion carriers, and 29 healthy controls. DNA was genotyped for GAA expansion, mRNA/FXN was quantified in real-time, and frataxin protein was measured using lateral-flow immunoassay in peripheral blood mononuclear cells (PBMCs). Mean residual levels of frataxin, compared to controls, were 35.8%, 65.6%, 33%, and 68.7% in cFA, LOFA, pFA and healthy carriers, respectively. Comparison of both cFA and pFA with controls resulted in 100% sensitivity and specificity, but there was overlap between LOFA, carriers and controls. Frataxin levels correlated inversely with GAA1 and GAA2 expansions, and directly with age at onset. Messenger RNA expression was reduced to 19.4% in cFA, 50.4% in LOFA, 52.7% in pFA, 53.0% in carriers, as compared to controls (p<0.0001). mRNA levels proved to be diagnostic when comparing cFA with controls resulting in 100% sensitivity and specificity. In cFA and LOFA patients mRNA levels correlated directly with protein levels and age at onset, and inversely with GAA1 and GAA2. CONCLUSION/SIGNIFICANCE: We report the first explorative study on combined frataxin and mRNA levels in PBMCs from a cohort of FRDA patients, carriers and healthy individuals. Lateral-flow immunoassay differentiated cFA and pFA patients from controls, whereas determination of mRNA in q-PCR was sensitive and specific only in cFA

The GAA triplet-repeat is unstable in the context of the human FXN locus and displays age-dependent expansions in cerebellum and DRG in a transgenic mouse model

Friedreich ataxia (FRDA) is caused by homozygosity for FXN alleles containing an expanded GAA triplet-repeat (GAA-TR) sequence. This expanded GAA-TR sequence is unstable in somatic cells of FRDA patients, showing age-dependent expansions in dorsal root ganglia (DRG), the tissue where pathology occurs earliest and is most significant. This is thought to be the basis for the progressive, tissue-specific pathology seen in FRDA, but the mechanism(s) for this somatic instability is unknown. We show that transgenic mice containing the expanded GAA-TR sequence (190 or 82 triplets) in the context of the human FXN locus show tissue-specific and age-dependent somatic instability that mimics the human condition. Small pool PCR analysis, which allows quantitative analysis of instability by assaying individual transgenes in vivo, showed age-dependent expansions specifically in the cerebellum and DRG. The (GAA)190 allele showed some instability by 2 months, progressed at about 0.3 – 0.4 triplets/week, resulting in a significant number of expansions by 12 months. Repeat length determined the age of onset of somatic instability, and the rate and magnitude of expansion. Whereas the GAA-TR was unstable in the context of the human FXN locus, pure GAATR sequences at other genetic loci in the human and murine genomes showed no instability. These data indicate that somatic instability of the GAA-TR sequence in the human FXN gene is determined by a combination of unique cis and trans-acting factors. This mouse model will serve as a useful tool to delineate the mechanism(s) of diseasespecific somatic instability in FRDA