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Many scholars point to the close association between early modern science and the rise of rational arguments in favour of the existence of witches. For some commentators, it is a poor reflection on science that its methods so easily lent themselves to the unjust persecution of innocent men and women. In this paper, I examine a debate about witches between a woman philosopher, Margaret Cavendish (1623-1673), and a fellow of the Royal Society, Joseph Glanvill (1636-1680). I argue that Cavendish is the voice of reason in this exchange—not because she supports the modern-day view that witches do not exist, but because she shows that Glanvill’s arguments about witches betray his own scientific principles. Cavendish’s responses to Glanvill suggest that, when applied consistently, the principles of early modern science could in fact promote a healthy scepticism toward the existence of witches.  相似文献   
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Mutations involving gains of glycosylation have been considered rare, and the pathogenic role of the new carbohydrate chains has never been formally established. We identified three children with mendelian susceptibility to mycobacterial disease who were homozygous with respect to a missense mutation in IFNGR2 creating a new N-glycosylation site in the IFNgammaR2 chain. The resulting additional carbohydrate moiety was both necessary and sufficient to abolish the cellular response to IFNgamma. We then searched the Human Gene Mutation Database for potential gain-of-N-glycosylation missense mutations; of 10,047 mutations in 577 genes encoding proteins trafficked through the secretory pathway, we identified 142 candidate mutations ( approximately 1.4%) in 77 genes ( approximately 13.3%). Six mutant proteins bore new N-linked carbohydrate moieties. Thus, an unexpectedly high proportion of mutations that cause human genetic disease might lead to the creation of new N-glycosylation sites. Their pathogenic effects may be a direct consequence of the addition of N-linked carbohydrate.  相似文献   
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Warburg Micro syndrome (WARBM1) is a severe autosomal recessive disorder characterized by developmental abnormalities of the eye and central nervous system and by microgenitalia. We identified homozygous inactivating mutations in RAB3GAP, encoding RAB3 GTPase activating protein, a key regulator of the Rab3 pathway implicated in exocytic release of neurotransmitters and hormones, in 12 families with Micro syndrome. We hypothesize that the underlying pathogenesis of Micro syndrome is a failure of exocytic release of ocular and neurodevelopmental trophic factors.  相似文献   
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Résumé Des organes de plantes ou d'animaux mis dans une suspension bactérienne synthétisent du RNA bactérien. Ce phénomène que nous avons appelé transcession est dû au transfert de DNA spontanément cédé par les bactéries vivantes aux cellules des organismes supérieurs. Dans le présent travail, il est démontré que le même phénomène peut avoir lieu naturellement lorsqu'une grenouille est sujette à une infection bactérienne.

We thank MissA. Cattaneo, MissJ. Henry for excellent technical assistance. This work was supported by a grant from the Fonds National Suisse de la Recherche Scientifique.  相似文献   
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An approach is presented for analyzing a heterogeneous set of categorical variables assumed to form a limited number of homogeneous subsets. The variables generate a particular set of proximities between the objects in the data matrix, and the objective of the analysis is to represent the objects in lowdimensional Euclidean spaces, where the distances approximate these proximities. A least squares loss function is minimized that involves three major components: a) the partitioning of the heterogeneous variables into homogeneous subsets; b) the optimal quantification of the categories of the variables, and c) the representation of the objects through multiple multidimensional scaling tasks performed simultaneously. An important aspect from an algorithmic point of view is in the use of majorization. The use of the procedure is demonstrated by a typical example of possible application, i.e., the analysis of categorical data obtained in a free-sort task. The results of points of view analysis are contrasted with a standard homogeneity analysis, and the stability is studied through a Jackknife analysis.  相似文献   
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Systemic Practice and Action Research - This article proposes a model based on the integration of systemic mechanisms such as the soft systems methodology, partial least squares path modelling and...  相似文献   
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