Early eighteenth-century Newtonianism: the Huguenot contribution

John Theophilus Desaguliers’s allegorical poem The Newtonian system of the world, the best model of government (1728) crystallizes the contribution of several important French Protestant exiles to the construction of early Newtonianism. In the context of diverging interpretations of Newton’s scientific achievement in terms of natural religion, writers such as Des Maizeaux, Coste, Le Clerc and others actively disseminated a version of Newtonianism which was close to Newton’s own intention. Through public experiments, translations, correspondence, reviews and books, they managed to convey a vision of Newtonian science which coincided with their propaganda of English liberties in Church and State. Therefore their effort on behalf of Newtonianism can be interpreted as part of a wider strategy of assimilation into English society at a time when most exiled Huguenots had given up hope of ever recreating a French Reformed Church at home.     

Producing objects, producing texts: accounts of android automata in late eighteenth-century Europe

Using the famous android automata made by the Swiss clockmakers Jaquet-Droz as an example, this essay examines the relationship between the production of automata and the production of texts about them against the background of economic and cultural conditions of artisan production and of the emerging ‘media industry’ in the public sphere of the late eighteenth century on the European continent. It explores the artisan environment in which the automata came into being and develops readings of various types of texts on the automata, including analyses of the contexts and print media of which they were a part. The essay concludes by explaining two interrelated peculiarities about eighteenth-century texts on the Jaquet-Droz automata: the fact that none of the texts displayed any interest in the metaphysical or ethical consequences of artificial or mechanical humans, and that they were, on the contrary, often simply copied from each other and thus adhered to the customs and exigencies of the emerging media industry of the time. The pre-industrial, traditional, non-mass produced construction of the automata thus coincided with the emerging contemporary mass production of texts and media, and it makes these texts and their objects bring into focus a key paradox underlying the history of modernity, the mass production of individuals and of individuality.     

From natural history to political economy: the enlightened mission of Domenico Vandelli in late eighteenth-century Portugal

This article presents the main features of the work of Domenico Vandelli (1735–1816), an Italian-born man of science who lived a large part of his life in Portugal. Vandelli’s scientific interests as a naturalist paved the way to his activities as a reformer and adviser on economic and financial issues. The topics covered in his writings are similar to those discussed by Linnaeus, with whom Vandelli corresponded. They clearly reveal that the scientific preparation indispensable for a better knowledge of natural resources was also a fundamental condition for correctly addressing problems of efficiency in their economic allocation. The key argument put forward in this article is that the relationship between natural history and the agenda for economic reform and development deserves to be further analysed. It is indeed a central element in the emergence of political economy as an autonomous scientific discourse during the last decades of the eighteenth century.     

The London evening courses of Benjamin Martin and James Ferguson,eighteenth-century lecturers on experimental philosophy

A study of some London newspapers of the early 1770s has shown that Martin and Ferguson gave continuous courses of evening lectures during the winter, in direct competition with each other. In this paper the coverage of their courses is derived from their advertisements, and related to their publications and other activities. In some subjects, such as Electricity, Hydrostatics, and Air-pump Experiments, there was close correspondence between the courses, but others reflected the lecturers' primary interests: for Martin, Optics, and for Ferguson, Mechanics and Astronomy.     

Glycosylation defects in inherited muscle disease

The gene mutated in the myodystrophy mouse, a model of muscular dystrophy, encodes a putative glycosyltransferase, Large. Mutations in genes encoding proteins thought to be involved in glycosylation have now been identified in six human forms of muscular dystrophy. Hereditary inclusion body myopathy and Nonaka myopathy result from defects in sialic acid production. Two forms of congenital muscular dystrophy, Fukuyama-type and MDC1C, result from mutations in members of the fukutin family. MDC1C and limb girdle muscular dystrophy type 2I are allelic, as they are both associated with mutations in the FKRP gene. Mutations in POMGnT, which encodes an enzyme involved in the synthesis of O-mannosyl glycans, result in muscle-eye-brain disease--another congenital form of muscular dystrophy. Abnormal alpha-dystroglycan has been reported in the myodystrophy mouse, and in the congenital and limb girdle muscular dystrophies. Recent data have shown that there is altered glycosylation of the protein and that this reduces its ability to bind to extracellular matrix ligands such as laminin and agrin.     

The wedge and the vis viva controversy: how concepts of force influenced the practice of early eighteenth-century mechanics

This article discusses the quest for the mechanical advantage of the wedge in the eighteenth century. As a case study, the wedge enlightens our understanding of eighteenth-century mechanics in general and the controversy over “force” or vis viva in particular. In this article, I show that the two different approaches to mechanics, the one that favoured force in terms of velocities and the one that primarily used displacements—known as the ‘Newtonian’ and ‘Leibnizian’ methods, respectively—were not at all on par in their ability to solve the problem of the wedge. In general, only those who used the Leibnizian concept of force or some related notion were able to get to the conventional results. This article thus rebuts the received view that the vis viva controversy was merely a semantic one. Instead, it shows that different understandings of “force” led to real and pragmatic differences in eighteenth-century mechanics.     

Endothelial defects and blood flow disturbance in atherogenesis

Résumé Les observations faites sur le porc de Yorkshire montrent que certaines perturbations du flot sanguin normal se manifestent dans une zone prédisposée au developpement de lésions athéromateuses et sont accompagnées de pertes de substances endothéliales. Cette destruction des cellules endothéliales permettrait au plasma riche en lipoprotéines de pénétrer librement dans la paroi artérielle, ce qui favorise l'athérogénese.

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Supported by NIH grant No. HE 11-11791.     

Malignant hyperthermia: Molecular defects in membrane permeability

Summary Malignant hyperthermia (MH), a genetically inherited disorder of skeletal muscle, is due to molecular defect in membrane permeability. The alteration in membrane permeability is suggested to be due to enhanced phospholipase A₂ activity which is responsible for the increased level in sarcoplasmic Ca²⁺. The excess Ca²⁺ is responsible for muscle hyper-rigidity and enhanced rate of glycolysis, resulting in a rapid rate of lactic acid production and a low pH in MH muscle.     

Microtubule transport defects in neurological and ciliary disease

Microtubules are primarily responsible for facilitating long-distance transport of both proteins and organelles. Given the critical role of this process in cellular function, it is not surprising that perturbation of microtubule-based transport can lead to diverse phenotypes in humans, including cancer and neurodegenerative disorders such as Alzheimer or Huntington disease. Recent investigations have also indicated that defects in specialized microtubule-based transport systems, such as mutations affecting the transport of protein particles along the length of cilia (intraflagellar transport) can cause retinal dystrophy, polycystic kidney disease or more complex syndromic phenotypes, such as Bardet-Biedl syndrome. In this review, we discuss recent findings implicating defects in microtubule-associated transport and motor proteins in a variety of diseases, particularly the role of defective microtubular transport in neurological and ciliary disease. These defects frequently display phenotypic consequences that manifest as human disease yet do not cause organismal lethality.Received 7 Janury 2005; received after revision 23 February 2005; accepted 21 March 2005     

Malignant hyperthermia: molecular defects in membrane permeability

Malignant hyperthermia (MH), a genetically inherited disorder of skeletal muscle, is due to molecular defect in membrane permeability. The alteration in membrane permeability is suggested to be due to enhanced phospholipase A2 activity which is responsible for the increased level in sarcoplasmic Ca2+. The excess Ca2+ is responsible for muscle hyper-rigidity and enhanced rate of glycolysis, resulting in a rapid rate of lactic acid production and a low pH in MH muscle.     

Mitochondrial defects and hearing loss

The techniques of human molecular genetics have been rapidly applied to the study of hearing loss. These studies have implicated more than 60 loci as causes of nonsyndromic hearing loss. Mutations at more than a dozen nuclear genes have been demonstrated to cause hearing loss, and these have been covered in recent reviews. However, a perhaps unexpected feature of the molecular characterization of human hearing loss has been the occurrence of mutations in the mitochondrial DNA (mtDNA). The importance of mitochondrial function in hearing is emphasized by the recent discovery of mutations in a nuclear-encoded mitochondrial protein which results in hearing loss. This article reviews the current status of our knowledge of mtDNA mutations that have been shown to cause hearing loss, and the suggestion of potential molecular, cellular and tissue-specific pathophysiological mechanisms by which dysfunction of mitochondria may lead to a loss of hearing.     

Phospholipase C-induced neural tube defects in the mouse embryo

Summary Mouse embryo neurulae were exposed in vitro to phospholipase C to examine the role of carbohydrate-rich extracellular material (ECM) during neurulation. Exposure of embryos to this agent for 12 h resulted in failure of closure of the neural tube. Ultrastructural examination revealed an absence of ECM from regions of the neural tube which failed to close.This study was supported by a grant from the National Fund for Research into Crippling Diseases.     

The late-twentieth century resolution of a mid-nineteenth century dilemma generated by the eighteenth-century experiments of Ernst Chladni on the dynamics of rods

Communicated by C. Truesdell     

Psychiatric behaviors associated with cytoskeletal defects in radial neuronal migration

Normal development of the cerebral cortex is an important process for higher brain functions, such as language, and cognitive and social functions. Psychiatric disorders, such as schizophrenia and autism, are thought to develop owing to various dysfunctions occurring during the development of the cerebral cortex. Radial neuronal migration in the embryonic cerebral cortex is a complex process, which is achieved by strict control of cytoskeletal dynamics, and impairments in this process are suggested to cause various psychiatric disorders. Our recent findings indicate that radial neuronal migration as well as psychiatric behaviors is rescued by controlling microtubule stability during the embryonic stage. In this review, we outline the relationship between psychiatric disorders, such as schizophrenia and autism, and radial neuronal migration in the cerebral cortex by focusing on the cytoskeleton and centrosomes. New treatment strategies for psychiatric disorders will be discussed.     

Genetic and developmental defects of the mouse corpus callosum

Summary Among adult BALB mice fewer than 20% usually have a small or absent corpus callosum (CC) and inheritance is polygenic. In the fetus at the time when the CC normally forms, however, almost all BALB mice show a distinct bulge in the interhemispheric fissure and grossly retarded commissure formation, and inheritance appears to result from two autosomal loci, provided the overall maturity of fetuses is equated. Most fetuses recover from the early defect when the CC axons manage to cross over the hippocampal commissure, and thus there is developmental compensation for a genetic defect rather than arrested midline development. The pattern of interhemispheric connections when the adult CC is very small is topographically normal in most respects, despite the unusual paths of the axons. The proportion of mice which fail to recover completely can be doubled by certain features of the maternal environment, and the severity of defects in adults can also be exacerbated by new genetic mutations which create new BALB substrains. The behavioral consequences of absent CC in mice are not known, nor have electrophysiological patterns been examined. The mouse provides an important model for prenatal ontogeny and cortical organization in human CC agenesis, because these data are not readily available for the human condition.     

Gaucher disease,a paradigm for single gene defects

Gaucher disease is the most common glycolipid storage disease. Type I, the most common form of the disease, is characterised by enlargement of the liver, and spleen and bone lesions. In the rare type II and type III forms of the disorder, central nervous system involvement is present as well. The disease results from a deficiency of the lysosomal enzyme glucocerebrosidase, which is needed for the enzymatic degradation of complex lipids, globosides and gangliosides. In the absence of sufficient glucocerebrosidase activity, the catabolic product glucocerebroside accumulates.     

Genetic and developmental defects of the mouse corpus callosum

Among adult BALB mice fewer than 20% usually have a small or absent corpus callosum (CC) and inheritance is polygenic. In the fetus at the time when the CC normally forms, however, almost all BALB mice show a distinct bulge in the interhemispheric fissure and grossly retarded commissure formation, and inheritance appears to result from two autosomal loci, provided the overall maturity of fetuses is equated. Most fetuses recover from the early defect when the CC axons manage to cross over the hippocampal commissure, and thus there is developmental compensation for a genetic defect rather than arrested midline development. The pattern of interhemispheric connections when the adult CC is very small is topographically normal in most respects, despite the unusual paths of the axons. The proportion of mice which fail to recover completely can be doubled by certain features of the maternal environment, and the severity of defects in adults can also be exacerbated by new genetic mutations which create new BALB substrains. The behavioral consequences of absent CC in mice are not known, nor have electrophysiological patterns been examined. The mouse provides an important model for prenatal ontogeny and cortical organization in human CC agenesis, because these data are not readily available for the human condition.