395 research outputs found

    A woman’s place is in the House, the Senate, just not the Judiciary? An empirical analysis of the relationship between a nominee’s gender and the Senate confirmation process

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    A rampant supposition exists that the judicial nominations of females are less successful due to the nominee’s gender (Martinek 2002). It is thus paramount to further investigate empirically whether individual nominee characteristics, such as gender, inhibit the nominee’s possibility of obtaining Senate confirmation. I empirically explore this conjecture in two distinct ways. First, I employ a difference in means test to determine whether women are confirmed to the District Court at a lower rate, on average, than are men. Subsequently, I test the hypothesis using a logistic regression that examines the influence of gender and the interaction of gender and race on the likelihood of confirmation, while controlling for other factors. Aiming to contribute to previous scholarship by providing an updated empirical analysis, I offer an update to Wendy Martinek’s original analysis of judicial confirmations by using the Lower Federal Court Confirmation Database to examine whether the influences of gender, race and their interaction on confirmation dynamics vary by partisan control. Having found the effect that nonwhite women are less likely to be confirmed by a GOP Senate than white males, I examine whether gender and race are the key factors or whether the relationship may instead be driven by ideology. Despite popular belief, the analysis of the data is not supportive of an extensive gender gap and undermines the claim that gender alone is an imperative factor inhibiting women from obtaining a successful confirmation. However, the empirical results are supportive of the hypothesis that racial minority females are less likely to be confirmed by a GOP controlled Senate than their white female and male counterparts

    Migrations climatiques: quel rĂŽle pour le droit international?

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    Ce papier analyse le rĂŽle des diffĂ©rentes branches du droit international concernĂ©es par les migrations climatiques : le droit international de l’environnement, le droit international des droits de l’homme, le droit international des rĂ©fugiĂ©s et le droit international des migrations. MĂȘme s’il ne porte formellement qu’une attention limitĂ©e aux migrations climatiques, le droit de l’environnement joue implicitement un rĂŽle significatif dans leur prĂ©vention en assurant la protection de l’environnement naturel. Les droits de l’homme, bien qu’ils nĂ©cessiteraient une application plus rigoureuse, jouent un rĂŽle important tant d’un point de vue normatif que prĂ©ventif dans la protection des migrants climatiques et ce de trois façons (dans le pays d’origine, dans le pays de destination et pour ce qui concerne le refoulement). Le droit des rĂ©fugiĂ©s est un « palliatif », assurant la protection des migrants forcĂ©s dont les droits ont Ă©tĂ© violĂ©s. Enfin, le droit de la migration, dans son Ă©tat actuel, est particuliĂšrement pertinent en ce qui concerne les droits que les migrants climatiques, devenus travailleurs migrants dans un pays Ă©tranger, sont en mesure de faire valoir dans le pays oĂč ils sont employĂ©s. MalgrĂ© leurs dĂ©fauts respectifs, ces quatre branches du droit international ont un rĂŽle spĂ©cifique et important eu Ă©gard Ă  la problĂ©matique des migrations climatiques. IsolĂ©ment, leur fonction est limitĂ©e, mais envisagĂ©es conjointement, elles forment un dispositif satisfaisant

    Prion protein in the cerebrospinal fluid of healthy and naturally scrapie-affected sheep

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    The aim of this study was to characterize the cerebrospinal fluid (CSF) prion protein (PrP) of healthy and naturally scrapie-affected sheep. The soluble form of CSF PrPC immunoblotted with an anti-octarepeat and an anti-C terminus mAb showed two isoforms of approximately 33 and 26 kDa, corresponding to the biglycosylated and unglycosylated isoforms of brain PrPC. Neither the mean concentration nor the electrophoretic profile of CSF PrP differed between healthy and scrapieaffected sheep, whereas a slightly increased resistance of CSF PrP to mild proteolysis by proteinase K was evident in the CSF of scrapie-affected sheep. No difference in susceptibility to proteolysis was observed between the two ARR and VRQ genetic variants of the purified prokaryote recombinant PrP. It was concluded that the physicochemical properties of PrPC in the CSF could be altered during scrapie and that these changes might reflect the physiopathological process of prion disease

    Evaluation of cytotoxic effects and acute and chronic toxicity of aqueous extract of the seeds of Calycotome villosa (Poiret) Link (subsp. intermedia) in rodents

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    Objective: The present investigation was carried out to evaluate the safety of an aqueous extract of the seeds of Calycotome villosa (Poiret) Link (subsp. intermedia) by determining its cytotoxicity and potential toxicity after acute and sub-chronic administration in rodents. Materials and Methods: Cytotoxic activity was tested in cancer and non-cancer cell lines HeLa, Mel-5, HL-60 and 3T3. Acute toxicity tests were carried out in mice by a single oral administration of Calycotome seed-extract (0 - 12 g/kg) as well as intraperitoneal doses of 0 - 5 g/kg.  Sub-chronic studies were conducted in Wistar rats by administration of oral daily doses for up to 90 days. Changes in body and vital organ weights, mortality, haematology, clinical biochemistry and histologic morphology were evaluated. Results: The lyophilized aqueous extract of C. villosa exhibited a low cytotoxicity in all cell lines tested with an IC50 > 100 ”g/ml. In the acute study in mice, intra-peritoneal administration caused dose-dependent adverse effects and mortality with an LD50 of 4.06 ± 0.01 g/kg.In the chronic tests, neither mortality nor visible signs of lethality was seen in rats. Even AST and ALT were not affected while a significant decrease in serum glucose levels, at 300 and 600 mg/kg was detected.  Histopathological examination of the kidney and liver did not show any alteration or inflammation at the end of treatment. Conclusion: In conclusion, the aqueous extract of C. villosa seed appeared to be non-toxic and did not produce mortality or clinically significant changes in the haematological and biochemical parameters in rats

    Dysregulation of oncogenic factors by GFI1B p32: investigation of a novel GFI1B germline mutation

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    GFI1B is a transcription factor essential for the regulation of erythropoiesis and megakaryopoiesis, and pathogenic variants have been associated with thrombocytopenia and bleeding. Analysing thrombocytopenic families by whole exome sequencing, we identified a novel GFI1B variant (c.648+5G>A), which causes exon 9 skipping and overexpression of a shorter p32 isoform. We report the clinical data of our patients and critically review the phenotype observed in individuals with different GFI1B variants leading to the same effect on the p32 expression. Since p32 is increased in acute and chronic leukemia cells, we tested the expression level of genes playing a role in various type of cancers, including hematological tumors and found that they are significantly dysregulated, suggesting a potential role for GFI1B in carcinogenesis regulation. Increasing the number of individuals with GFI1B variants will allow us to better characterize this rare disease and determine whether it is associated with an increased risk of developing malignancies

    Habiter le dedans et le dehors : la maison ou l’Eden rĂȘvĂ© et recrĂ©Ă©

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    Avec pour hypothĂšse commune que la montĂ©e actuelle de la « question habitante » dans les sociĂ©tĂ©s post industrielles oblige Ă  mettre au centre de l’investigation : . le point de vue de l’individu habitant, Ă  partir duquel il faut tenter de dĂ©finir les « cultures de la nature », les reprĂ©sentations de la valeur des lieux, les catĂ©gories par lesquelles chaque sujet pense sa « gĂ©ographicité » ; 2. les catĂ©gories de rural et d’urbain rendent possible la mise en Ă©vidence des propriĂ©tĂ©s et des qualitĂ©s de chaque milieu quel que soit le pays d'Europe concernĂ©. Les auteurs tentent de dĂ©gager des rĂ©sultats transversaux des rĂ©cits de lieux de vie obtenus dans leurs terrains spĂ©cifiques de recherche. Ils aboutissent Ă  une problĂ©matique collective et Ă  une mĂ©thodologie Ă  mettre en place pour approfondir ce qu’habiter veut dire aujourd’hui dans les reprĂ©sentations et les pratiques des ruraux et des urbains. La contradiction entre le chez soi pensĂ© et pratiquĂ© comme « espace refuge » ou « dedans » et le chez soi vu comme appropriation de l’abord et intĂ©gration de la discontinuitĂ© du dehors est un principe de distinction fort pour aller vers une typologie des « modes d’habiter ».The authors base their reflection on the common assumption that the growing importance of the “living issue” in post-industrial societies compels them to focus their investigation on: 1) the point of view of the resident, from which they must try to define the “cultures of nature”, the representations of the value of the place and the categories through which each individual relates to his “geographicity”; 2) the categories of the rural and the urban whose confrontation is likely to evidence the qualities and properties of any European-based “milieu”. The authors then synthetise the data provided by the narratives collected in their own fields of investigation. They can thus formulate a common question and methodology in order to reflect on what “living” means today in the representations and practices of country people and city dwellers. The contradiction between the home considered and experienced as a shelter or “inside” and the home seen as an appropriation of the margins and integration of the discontinuity of the “outside” is a strong distinctive principle to head towards a typology of the “ways of living”

    Habiter le dedans et le dehors : la maison ou l’Eden rĂȘvĂ© et recrĂ©Ă©

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    Avec pour hypothĂšse commune que la montĂ©e actuelle de la « question habitante » dans les sociĂ©tĂ©s post industrielles oblige Ă  mettre au centre de l’investigation : . le point de vue de l’individu habitant, Ă  partir duquel il faut tenter de dĂ©finir les « cultures de la nature », les reprĂ©sentations de la valeur des lieux, les catĂ©gories par lesquelles chaque sujet pense sa « gĂ©ographicité » ; 2. les catĂ©gories de rural et d’urbain rendent possible la mise en Ă©vidence des propriĂ©tĂ©s et des qualitĂ©s de chaque milieu quel que soit le pays d'Europe concernĂ©. Les auteurs tentent de dĂ©gager des rĂ©sultats transversaux des rĂ©cits de lieux de vie obtenus dans leurs terrains spĂ©cifiques de recherche. Ils aboutissent Ă  une problĂ©matique collective et Ă  une mĂ©thodologie Ă  mettre en place pour approfondir ce qu’habiter veut dire aujourd’hui dans les reprĂ©sentations et les pratiques des ruraux et des urbains. La contradiction entre le chez soi pensĂ© et pratiquĂ© comme « espace refuge » ou « dedans » et le chez soi vu comme appropriation de l’abord et intĂ©gration de la discontinuitĂ© du dehors est un principe de distinction fort pour aller vers une typologie des « modes d’habiter ».The authors base their reflection on the common assumption that the growing importance of the “living issue” in post-industrial societies compels them to focus their investigation on: 1) the point of view of the resident, from which they must try to define the “cultures of nature”, the representations of the value of the place and the categories through which each individual relates to his “geographicity”; 2) the categories of the rural and the urban whose confrontation is likely to evidence the qualities and properties of any European-based “milieu”. The authors then synthetise the data provided by the narratives collected in their own fields of investigation. They can thus formulate a common question and methodology in order to reflect on what “living” means today in the representations and practices of country people and city dwellers. The contradiction between the home considered and experienced as a shelter or “inside” and the home seen as an appropriation of the margins and integration of the discontinuity of the “outside” is a strong distinctive principle to head towards a typology of the “ways of living”

    A 2.3-Day Periodic Variability in the Apparently Single Wolf-Rayet Star WR 134: Collapsed Companion or Rotational Modulation?

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    We present the results of an intensive campaign of spectroscopic and photometric monitoring of the peculiar Wolf-Rayet star WR 134 from 1989 to 1997. This unprecedentedly large data set allows us to confirm unambiguously the existence of a coherent 2.25 +/- 0.05 day periodicity in the line-profile changes of He II 4686, although the global pattern of variability is different from one epoch to another. This period is only marginally detected in the photometric data set. Assuming the 2.25 day periodic variability to be induced by orbital motion of a collapsed companion, we develop a simple model aiming at investigating (i) the effect of this strongly ionizing, accreting companion on the Wolf-Rayet wind structure, and (ii) the expected emergent X-ray luminosity. We argue that the predicted and observed X-ray fluxes can only be matched if the accretion on the collapsed star is significantly inhibited. Additionally, we performed simulations of line-profile variations caused by the orbital revolution of a localized, strongly ionized wind cavity surrounding the X-ray source. A reasonable fit is achieved between the observed and modeled phase-dependent line profiles of He II 4686. However, the derived size of the photoionized zone substantially exceeds our expectations, given the observed low-level X-ray flux. Alternatively, we explore rotational modulation of a persistent, largely anisotropic outflow as the origin of the observed cyclical variability. Although qualitative, this hypothesis leads to greater consistency with the observations.Comment: 34 pages, 16 figures. Accepted by the Astrophysical Journa

    Lentivirus-mediated gene therapy for Fabry disease

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    Enzyme and chaperone therapies are used to treat Fabry disease. Such treatments are expensive and require intrusive biweekly infusions; they are also not particularly efficacious. In this pilot, single-arm study (NCT02800070), five adult males with Type 1 (classical) phenotype Fabry disease were infused with autologous lentivirus-transduced, CD34+-selected, hematopoietic stem/progenitor cells engineered to express alpha-galactosidase A (α-gal A). Safety and toxicity are the primary endpoints. The non-myeloablative preparative regimen consisted of intravenous melphalan. No serious adverse events (AEs) are attributable to the investigational product. All patients produced α-gal A to near normal levels within one week. Vector is detected in peripheral blood and bone marrow cells, plasma and leukocytes demonstrate α-gal A activity within or above the reference range, and reductions in plasma and urine globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3) are seen. While the study and evaluations are still ongoing, the first patient is nearly three years post-infusion. Three patients have elected to discontinue enzyme therapy
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