34 research outputs found

    Constructing legitimacy?

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    How are agroecological transitions and rural development alternatives experienced by farmers and rural activists? To explore this broad issue, Claire Lagier examines how agroecology’s legitimacy is constructed and contested within the base membership and transnational networks of Brazil’s Landless Workers’ Movement (MST). The MST’s politics of land redistribution—as well as campaigning for sustainable rural livelihoods and education—have attracted significant attention worldwide, as have the food sovereignty alliance La Via Campesina’s agroecological training centres. However, few ethnographic studies have focused on the lived experiences of several generations of activists as they struggle to generate ecological transitions in the food system. Addressing this gap, Lagier’s study draws on intensive fieldwork carried out in Brazil in 2017–2018 alongside farmers living in a settlement affiliated with the MST, as well as young Latin American food sovereignty activists taking part in agroecological education. Claire Lagier defended her PhD thesis in Environmental Humanities at LMU Munich's Rachel Carson Center for Environment and Society in June 2019. Her doctoral work was funded by ENHANCE Marie-Curie ITN (2015-2018). She is currently based in Rio de Janeiro, Brazil

    Progressive dementia associated with ataxia or obesity in patients with Tropheryma whipplei encephalitis

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    <p>Abstract</p> <p>Background</p> <p><it>Tropheryma whipplei</it>, the agent of Whipple's disease, causes localised infections in the absence of histological digestive involvement. Our objective is to describe <it>T. whipplei </it>encephalitis.</p> <p>Methods</p> <p>We first diagnosed a patient presenting dementia and obesity whose brain biopsy and cerebrospinal fluid specimens contained <it>T. whipplei </it>DNA and who responded dramatically to antibiotic treatment. We subsequently tested cerebrospinal fluid specimens and brain biopsies sent to our laboratory using <it>T. whipplei </it>PCR assays. PAS-staining and <it>T. whipplei </it>immunohistochemistry were also performed on brain biopsies. Analysis was conducted for 824 cerebrospinal fluid specimens and 16 brain biopsies.</p> <p>Results</p> <p>We diagnosed seven patients with <it>T. whipplei </it>encephalitis who demonstrated no digestive involvement. Detailed clinical histories were available for 5 of them. Regular PCR that targeted a monocopy sequence, PAS-staining and immunohistochemistry were negative; however, several highly sensitive and specific PCR assays targeting a repeated sequence were positive. Cognitive impairments and ataxia were the most common neurologic manifestations. Weight gain was paradoxically observed for 2 patients. The patients' responses to the antibiotic treatment were dramatic and included weight loss in the obese patients.</p> <p>Conclusions</p> <p>We describe a new clinical condition in patients with dementia and obesity or ataxia linked to <it>T. whipplei </it>that may be cured with antibiotics.</p

    RRM adjacent TARDBP mutations disrupt RNA binding and enhance TDP-43 proteinopathy

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    Amyotrophic lateral sclerosis (ALS) presents with focal muscle weakness due to motor neuron degeneration that becomes generalized,leading to death from respiratory failure within 3–5 years from symptom onset. Despite the heterogeneity of aetiology, TDP- 43 proteinopathy is a common pathological feature that is observed in 495% of ALS and tau-negative frontotemporal dementia(FTD) cases. TDP-43 is a DNA/RNA-binding protein that in ALS and FTD translocates from being predominantly nuclear to formdetergent-resistant, hyperphosphorylated aggregates in the cytoplasm of affected neurons and glia. Mutations in TARDBP accountfor 1–4% of all ALS cases and almost all arise in the low complexity C-terminal domain that does not affect RNA binding andprocessing. Here we report an ALS/FTD kindred with a novel K181E TDP-43 mutation that is located in close proximity to the RRM1 domain. To offer predictive gene testing to at-risk family members, we undertook a series of functional studies to characterizethe properties of the mutation. Spectroscopy studies of the K181E protein revealed no evidence of significant misfolding.Although it is unable to bind to or splice RNA, it forms abundant aggregates in transfected cells. We extended our study to includeother ALS-linked mutations adjacent to the RRM domains that also disrupt RNA binding and greatly enhance TDP-43 aggregation,forming detergent-resistant and hyperphosphorylated inclusions. Lastly, we demonstrate that K181E binds to, and sequesters, wild-type TDP-43 within nuclear and cytoplasmic inclusions. Thus, we demonstrate that TDP-43 mutations that disrupt RNAbinding greatly enhance aggregation and are likely to be pathogenic as they promote wild-type TDP-43 to mislocalize andaggregate acting in a dominant-negative manner. This study highlights the importance of RNA binding to maintain TDP-43solubility and the role of TDP-43 aggregation in disease pathogenesis

    Agroecology within and beyond the Brazilian Landless Workers' Movement (MST) - Appendix 1 - 4

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    Appendix 1: This is a copy of the agreement between the state government of Paraná (Brazil), the Federal University of Paraná, the Venezuelan government, the Venezuelan state government of Yaracuy and La Via Campesina International to create an agroecology training course in Paraná and in Venezuela. Signed on January 30th, 2005 in Tapes, Brazil. Photographed by Claire Lagier in May 2017. Contains two pages. Original title (Spanish): "Protocolo de intenciones y compromisos que hacen entre sí: La Via Campesina, el gobierno de la Republica Bolivariana de Venezuela, el gobierno del estado de Paraná y instituciones de enseñanza del Brasil y de Venezuela". This document is cited in Claire Lagier's thesis (DOI https://doi.org/10.5282/oph.8), Chapter 5.Appendix 2: This is the original flowchart document handed in 2017 to students enrolled in the Bachelor of Agroecology course at the Ecological Resistance School (this is a pseudonym) in Paraná, Brazil as part of the pedagogical activity named "Dialogue of knowledges in the encounter of cultures" by the individual I refer to as João Maria (also a pseudonym) in my thesis. This person is the original author of the document but I refrain from publishing their real name for research ethics reasons, in order to protect their anonymity. This copy is identical to the original except that I redacted João Maria's real name, the real name of Ecological Resistance School, and the real name of the municipality where it is located. Original title (Portuguese): “Fluxograma orientador ao diálogo de saberes no encontro de culturas". This document is analyzed and cited in my thesis (DOI https://doi.org/10.5282/oph.8), Chapters 5-7.Appendix 3: This is the original fieldwork guidelines document handed in 2017 to students enrolled in the Bachelor of Agroecology course at the Ecological Resistance School (this is a pseudonym) in Paraná, Brazil as part of the pedagogical activity named "Dialogue of knowledges in the encounter of cultures" by the individual I refer to as João Maria (also a pseudonym) in my thesis. This person is the original author of the document but I refrain from publishing their real name for research ethics reasons, in order to protect their anonymity. This copy is identical to the original except that I redacted João Maria's real name, the real name of Ecological Resistance School, and the real name of the rural settlement and municipality where it is located. Original title(Portuguese): “Roteiro orientador ao diálogo de saberes no encontro de culturas". This document is analyzed and cited in my thesis (DOI https://doi.org/10.5282/oph.8), Chapters 5-7.Appendix 4: This is the original report redaction guidelines document handed in 2017 to students enrolled in the Bachelor of Agroecology course at the Ecological Resistance School (this is a pseudonym) in Paraná, Brazil as part of the pedagogical activity named "Dialogue of knowledges in the encounter of cultures" by the individual I refer to as João Maria (also a pseudonym) in my thesis. This person is the original author of the document but I refrain from publishing their real name for research ethics reasons, in order to protect their anonymity. This copy is identical to the original except that I redacted João Maria's real name, the real name of Ecological Resistance School, and the real name of the rural settlement and municipality where it is located. Original title (Portuguese): “Roteiro para a sistematização do relatório do diálogo de saberes no encontro de culturas". This document is analyzed and cited in my thesis (DOI https://doi.org/10.5282/oph.8), Chapters 5-7.Zur Publikation: Lagier, Claire: Constructing legitimacy? Agroecology within and beyond the Brazilian Landless Workers' Movement (MST). München, Universitätsbibliothek Ludwig-Maximilians-Universität, 2020. ISBN (Druck) 978-3-95925-152-5. - ISBN (Online): 978-3-95925-153-2. Online-Version: https://doi.org/10.5282/oph.8 unter Creative Commons Lizenz CC BY 4.

    Etude posturale de sujets normaux en situation expérimentale d’effort vocal

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    International audienceIntroduction: The objective of the study was to assess how posture and vocal acoustics are modified when one needs to communicate despite environmental obstacles (i.e. vocal effort performance).Method: 20 healthy young women participated to this study. They had to communicate numbers to a listener. Environmental obstacles were the distance between the subject and the auditor, and the noise. Segmental posture modifications were recorded and analyzed using the numerical infra-red system for movement analysis SMART®. Vocal acoustics and electroglottography were also recorded and analyzed using the EVA® system.Results: The study showed the presence of objective postural modifications during vocal effort. Their amplitude increased when the environmental obstacles to communications increased, and they anticipated the vocal production.Discussion: The vocal effort concept and its relation with the posture, the voice and the subject’s feeling are reviewed on the basis of these results. Laryngeal effort is not isolated, but belongs to a global behavioral strategy which aims to improving the efficiency of the communication.Introduction : L’objectif de l’étude était d’évaluer les modifications posturales et vocales au cours d’une tâche de communication en environnement défavorable (effort vocal).Matériels et méthodes : Cette étude expérimentale a analysé le comportement vocal et postural de 20 femmes de 20 à 43 ans indemnes de problèmes posturaux, vocaux ou auditifs. Les sujets devaient dicter des nombres à un auditeur. Les conditions défavorables à la communication étaient la distance entre le sujet et l’auditeur, et le bruit ambiant. Les modifications posturales segmentaires ont été enregistrées à l’aide du système numérique infrarouge d’analyse du mouvement SMART®. Les modifications vocales étaient évaluées par des paramètres acoustiques objectifs et par électroglottographie à l’aide de la station EVA®.Résultats : L’étude a montré l’existence de modifications posturales contemporaines de l’effort vocal. Ces modifications posturales sont d’autant plus amples que l’environnement est plus défavorable (donc que l’effort vocal est plus important), et le mouvement anticipe la production vocale.Discussion : Le concept de forçage vocal et ses relations avec la posture, la voix et le ressenti du sujet sont revus sur la base de ces résultats. L’effort laryngé n’est pas seul impliqué dans l’effort vocal, il fait partie d’une stratégie comportementale globale visant à améliorer l’efficacité de la communication

    Use of SNP array analysis to identify a novel TRIM32 mutation in limb-girdle muscular dystrophy type 2H.

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    International audienceMolecular diagnosis of monogenic diseases with high genetic heterogeneity is usually challenging. In the case of limb-girdle muscular dystrophy, multiplex Western blot analysis is a very useful initial step, but that often fails to identify the primarily affected protein. We report how homozygosity analysis using a genome-wide SNP array allowed us to solve the diagnostic enigma in a patient with a moderate form of LGMD, born from consanguineous parents. The genome-wide scan performed on the patient's DNA revealed several regions of homozygosity, that were compared to the location of known LGMD genes. One such region indeed contained the TRIM32 gene. This gene was previously found mutated in families with limb-girdle muscular dystrophy type 2H (LGMD2H), a mild autosomal recessive myopathy described in Hutterite populations and in 4 patients with a diagnosis of sarcotubular myopathy. A single missense mutation was found in all these patients, located in a conserved domain of the C-terminal part of the protein. Another missense mutation affecting the N-terminal part of TRIM32, observed in a single consanguineous Bedouin family, was reported to cause the phenotypically unrelated and genetically heterogeneous Bardet-Biedl syndrome, defining the BBS11 locus. Sequencing of TRIM32 in our patient revealed a distal frameshift mutation, c.1753_1766dup14 (p.Ile590Leu fsX38). Together with two recently reported mutations, this novel mutation confirms that integrity of the C-terminal domain of TRIM32 is necessary for muscle maintenance
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