Ten percent of cases of intellectual deficiency in boys are caused by genes located on the X chromosome. X-linked mental retardation (XLMR) includes more than 200 syndromes and 80 genes identified to date. The fragile X syndrome is the most frequent syndrome, due to a dynamic mutation with a CGG triplet amplification. Mental retardation is virtually always present. Phonological and syntactic impairments are often combined with pragmatic language impairment and visuospatial reasoning difficulties. A minority fulfill the criteria for autism. In girls, the clinical expression of the complete mutation varies according to the X chromosome inactivation profile. Several XLMR occur as severe early onset encephalopathies: Lowe oculocerebrorenal syndrome, ATR-X syndrome (alpha thalassemia/mental retardation X-linked), Allan–Herdon–Dudley syndrome (MCT8 gene). Two genes, ARX (X-LAG; Partington syndrome) and MECP2 (Rett syndrome in females; mild MR with spastic diplegia/psychotic problems in males) are associated with various phenotypes, according to the mutation involved. Oligophrenine 1 (OPHN-1) gene mutations lead to vermal dysplasia. PQBP1 gene mutations (Renpenning syndrome) are responsible for moderate to severe mental deficiency, microcephaly, and small stature. Although some forms of XLMR are not very specific and the phenotype for each given gene is somewhat heterogeneous, a clinical diagnostic strategy is emerging.
Ten percent of cases of intellectual deficiency in boys are caused by genes located on the X chromosome. X-linked mental retardation (XLMR) includes more than 200 syndromes and 80 genes identified to date. The fragile X syndrome is the most frequent syndrome, due to a dynamic mutation with a CGG triplet amplification. Mental retardation is virtually always present. Phonological and syntactic impairments are often combined with pragmatic language impairment and visuospatial reasoning difficulties. A minority fulfill the criteria for autism. In girls, the clinical expression of the complete mutation varies according to the X chromosome inactivation profile. Several XLMR occur as severe early onset encephalopathies: Lowe oculocerebrorenal syndrome, ATR-X syndrome (alpha thalassemia/mental retardation X-linked), Allan–Herdon–Dudley syndrome (MCT8 gene). Two genes, ARX (X-LAG; Partington syndrome) and MECP2 (Rett syndrome in females; mild MR with spastic diplegia/psychotic problems in males) are associated with various phenotypes, according to the mutation involved. Oligophrenine 1 (OPHN-1) gene mutations lead to vermal dysplasia. PQBP1 gene mutations (Renpenning syndrome) are responsible for moderate to severe mental deficiency, microcephaly, and small stature. Although some forms of XLMR are not very specific and the phenotype for each given gene is somewhat heterogeneous, a clinical diagnostic strategy is emerging.
These twenty-four papers originated at a conference held in 1999 which was dedicated to Xenophon's writings and to the many areas of Greek life for which he is a major source. The contributions, which also reflect the problems of recreating a life that we have so few facts for, are divided into seven sections which discuss: Xenophon's life; Xenophon and Socrates; Xenophon and the barbarian world; Sparta; religion and politics; Anabasis ; Hellenica . These wide-ranging papers are specialised, often based on a close reading on Xenophon's texts, and not all of the Greek is translated. Eighteen papers, plus the introduction, are in English; the remaining papers are in Italian or German.
Aging within Transnational Families' is the first book to provide a multi-method approach to studying aging across borders. By asking how, why and to what extent do older Peruvians engage in transnational family ties and practices, the book enhances our knowledge about aging across borders. Drawing on the care circulation framework and the capacity and desire approach, it explores the motivations of older Peruvians’ transnational involvement as well as the factors influencing the scope and propensity of their cross-border practices. From a lifecourse perspective, the book asks how age relates to older Peruvian migrants’ integration into the host society and engagement in the sending of remittances and visits of family members in Peru. Exploring the prevalence and structuring features of family-related transnational practices against the backdrop of different migration regimes 'Aging within Transnational Families' shows how policies affect transnational family configurations and the role of older people within them.
Anthropology has long had a vexed relationship with literature, and nowhere has this been more acutely felt than in France, where most ethnographers, upon returning from the field, write not one book, but two: a scientific monograph and a literary account. In Far Afield—brought to English-language readers here for the first time—Vincent Debaene puzzles out this phenomenon, tracing the contours of anthropology and literature’s mutual fascination and the ground upon which they meet in the works of thinkers from Marcel Mauss and Georges Bataille to Claude Lévi-Strauss and Roland Barthes. The relationship between anthropology and literature in France is one of careful curiosity. Literary writers are wary about anthropologists’ scientific austerity but intrigued by the objects they collect and the issues they raise, while anthropologists claim to be scientists but at the same time are deeply concerned with writing and representational practices. Debaene elucidates the richness that this curiosity fosters and the diverse range of writings it has produced, from Proustian memoirs to proto-surrealist diaries. In the end he offers a fascinating intellectual history, one that is itself located precisely where science and literature meet.
In this comprehensive study of Olivier Messiaen's magnum opus, Saint François d'Assise, Vincent Perez Benitez examines the opera from both theological and musical-analytical perspectives to ask how Messiaen expresses his Catholic theology through his work. Benitez combines a close reading of the opera score with accounts from Messiaen's associates, studies of Messiaen's birdsong notebooks and other primary documents, and an examination of the religious, musical, poetic, and visual arts literature with which the composer was familiar to explore how the opera's harmonic language and sound-color relationships motivate its musical meaning and expression. Through his analysis of these diverse sources and comparisons of Saint François d'Assise with other works such as Berg's Wozzeck and Wagner's Parsifal, Benitez places Messiaen's compositional practice within larger musical perspectives and historical contexts.
Constructing Gardens, Cultivating the City is the first cultural history of major new parks developed in Paris in the late twentieth century, as part of the city's program of adaptive reuse of industrial spaces. Thanks to laws that gave the city more political autonomy, Paris's local government launched a campaign of park creation in the late 1970s that continued to the turn of the millennium. The parks in this book represent this campaign and illustrate different facets of their cultural and historical context. Archival research, interviews, and analyses of the parks reveal how postmodern debates about urban planning, the historic city, public space, and nature's presence in an urban setting influenced their designs. In sum, the city adopted the garden as a model for public parks, investing in complex, richly symbolic and representational spaces. These parks were intended to represent contemporary twists on traditional designs and serve local residents as much as they would contribute to Paris's role as a world city. The parks' development process often included points of conflict, pointing to differing views on what Parisian space should represent and fundamental contradictions between the characteristics of public space and the garden as it is traditionally defined. These parks demonstrate the ongoing cultivation of the city over time, in which transformed sites not only fulfil new functions but also engage with history and their surroundings to create new meaning. They stand for landscape as a form of signifying cultural production that directly engages with other art forms and ways of knowing. Just as the Luxembourg Gardens, the Tuileries, and the Buttes-Chaumont parks exemplify their eras' cultural dynamics, such parks as the Jardin Atlantique, Parc André-Citroën, and the Jardin des Halles express contemporary French culture within the archetypal space of their era, the city. Finally, they point the way to current trends in landscape architecture, such as citizen gardening and ecological initiatives.
Published to accompany a major exhibition of Jean-Baptiste-Camille Corot's paintings held in Paris and Ottawa during 1996, and forthcoming to New York. From nearly 3,000 paintings by this poetic 19th-century artist, the curators chose 163 works, which are reproduced here along with full art-historical discussions of each. Three major essays chronicle Corot's life and the development of his art; additional essays elucidate the subject of forgeries and describe the collecting of his works. Much original new scholarship is included along with a review of the scholarly literature, a concordance, and a chronology. 9.5x12.5"Annotation copyrighted by Book News, Inc., Portland, OR
The feast of Corpus Christi, one of the most solemn feasts of the Latin Church, can be traced to the Fourth Lateran Council in 1215 and its resolution of disputes over the nature of the Eucharist. The feast was first celebrated in Liège in 1246, thanks largely to the efforts of a religious woman, Juliana of Mont Cornillon, who not only popularized the feast, but also wrote key elements of an original office. This volume presents for the first time a complete set of source materials germane to the study of the feast of Corpus Christi. In addition to the multiple versions of the original Latin liturgy, a set of poems in Old French, and their English translations, the book includes complete transcriptions of the music associated with the feast. An introductory essay lays out the historical context for understanding the initiation and reception of the feast.
Dotawo: A Journal of Nubian Studies offers a platform in which the old meets the new, in which archaeological, papyrological, and philological research into Meroitic, Old Nubian, Coptic, Greek, and Arabic sources confront current investigations in modern anthropology and ethnography, Nilo-Saharan linguistics, and critical and theoretical approaches present in post-colonial and African studies. Dotawo gives a common home to the past, present, and future of one of the richest areas of research in African studies. It offers a crossroads where papyrus can meet internet, scribes meet critical thinkers, and the promises of growing nations meet the accomplishments of old kingdoms.The third volume of Dotawo, guest-edited by Marc Maillot, is dedicated to Know-Hows and Techniques in Ancient Sudan. This collection of articles is the result of a workshop held at Lille University on September 5 and 6, 2013, which brought together several Sudanese archaeology scholars, from architecture to iron production through pottery and textile industry. Organized by Faïza Drici, Marie Evina, and Romain David, with the support of Charles de Gaulle-Lille 3 University and the laboratoire de recherche Halma-Ipel UMR 8164 (Centre national de recherche scientifique - CNRS), this workshop was presided over by Vincent Rondot (present Director of the Egyptian Antiquities Department of the Louvre Museum and former Director of Section française de la direction des antiquités du Soudan - SFDAS). The idea of an academic publication of this workshop in Dotawo was presented by Marc Maillot (SFDAS) in September 2014, during the 13th International Conference for Nubian Studies. The project was warmly welcomed by the editorial committee, and gave birth to a fruitful SFDAS/Dotawo cooperation that started a year ago.
L'an dernier, dans notre livre « Crise du système impérialiste mondial ― La décomposition finale de "l'industrie de bazar" et la naissance d'un nouvel ordre impérialiste mondial », nous avions souligné le caractère structurel de la crise actuelle, ainsi que le différentiel croissant entre l'impérialisme chinois et ses plus puissants concurrents. Nous avions également souligné que les résultats du plan de relance chinois contrasteraient nettement avec ceux de ses concurrents. Nous avions enfin souligné l'inéluctabilité de l'aggravation du déficit des Etats bourgeois qui seraient en définitive forcés de faire retomber le poids de la crise sur les masses exploitées au moyens de PAS (qualifiés de "plans de rigueur"), visant à la destruction des chaînes dorées de l'esclavage salarié. Les derniers mois ont confirmé toutes ces anticipations, comme l'illustre ce livre qui fait le point sur la rapide montée en puissance de l'impérialisme chinois et ses implications actuelles et futures.
The Roman orientation was the keystone of the religious revolution of the Catholic revival. New or renewed congregations, priests close to the people & militant laymen gave a decidedly social & activist turn to the faith. At this crossroad of religion & modernity, the papacy could all the more make its weight felt as the Belgian Constitution granted the clergy a unique liberty in relations with Rome. Over time, the Vatican would exert a powerful impact on the shape of modern politics in Belgium. The special relationship between Belgium & Rome was no one-way traffic. From a somewhat curious ecclesiastical court hopelessly entangled in the old spider web of the Papal States, the papacy became the institution we know today, the leader of a "modern" Catholic opinion. Belgium played a role of major importance in this transformation. The central theme of the book can therefore be defined as a process of mutual integration, if not acculturation, across the Alps.
The book is on the shortlist of ICAS Book Prize 2023 Humanities The origins of modern Daoism can be traced to the Church of the Heavenly Master (Tianshidao), reputedly established by the formidable Zhang Daoling. In 142 CE, according to Daoist tradition, Zhang was visited by the Lord on High, who named him his vicar on Earth with the title Heavenly Master. The dispensation articulated an eschatological vision of saving initiates—the pure, those destined to become immortals— by enforcing a strict moral code. Under evolving forms, Tianshidao has remained central to Chinese society, and Daoist priests have upheld their spiritual allegiance to Zhang, their now divinized founder. This book tells the story of the longue durée evolution of the Heavenly Master leadership and institution. Later hagiography credits Zhang Daoling’s great-grandson, putatively the fourth Heavenly Master, with settling the family at Longhushan (Dragon and Tiger Mountain); in time his descendants—down to the present contested sixty-fifth Heavenly Master living in Taiwan— made the extraordinary claim of being able to transmit hereditarily the function of the Heavenly Master and the power to grant salvation. Over the next twelve centuries, the Zhangs turned Longhushan into a major holy site and a household name in the Chinese world, and constructed a large administrative center for the bureaucratic management of Chinese society. They gradually built the Heavenly Master institution, which included a sacred site; a patriarchal line of successive Heavenly Masters wielding vast monopolistic powers to ordain humans and gods; a Zhang lineage that nurtured talent and accumulated wealth; and a bureaucratic apparatus comprised of temples, training centers, and a clerical hierarchy. So well-designed was this institution that it remained stable for more than a millennium, far outlasting the longest dynasties, and had ramifications for every city and village in imperial China. In this ambitious work, Vincent Goossaert traces the Heavenly Master bureaucracy from medieval times to the modern Chinese nation-state as well as its expansion. His in-depth portraits of influential Heavenly Masters are skillfully embedded in a large-scale analysis of the institution and its rules, ideology, and vision of society.
An exploration of Francophone African literary imaginations and expressions through the lens of Afrofuturism Generally attributed to the Western imagination, science fiction is a literary genre that has expressed projected technological progress since the Industrial Revolution. However, certain fantastical elements in African literary expressions lend themselves to science fiction interpretations, both utopian and dystopian. When the concept of science is divorced from its Western, rationalist, materialist, positivist underpinnings, science fiction represents a broad imaginative space that supersedes the limits of this world. Whether it be on the moon, under the sea, or elsewhere within the imaginative universe, Afrofuturist readings of select films, novels, short stories, plays, and poems reveal a similarly emancipatory African future that is firmly rooted in its own cultural mythologies, cosmologies, and philosophies. Isaac Joslin identifies the contours and modalities of a speculative, futurist science fiction rooted in the sociocultural and geopolitical context of continental African imaginaries. Constructing an arc that begins with gender identity and cultural plurality as the bases for an inherently multicultural society, this project traces the essential role of language and narrativity in processing traumas that stem from the violence of colonial and neocolonial interventions in African societies. Joslin then outlines the influential role of discursive media that construct divisions and create illusions about societal success, belonging, and exclusion, while also identifying alternative critical existential mythologies that promote commonality and social solidarity. The trajectory proceeds with a critical analysis of the role of education in affirming collective identity in the era of globalization; the book also assesses the market-driven violence that undermines efforts to instill and promote cultural and social autonomy. Last, this work proposes an egalitarian and ecological ethos of communal engagement with and respect for the diversity of the human and natural worlds.
Bugliosi, brilliant prosecutor and bestselling author, is perhaps the only man in America capable of "prosecuting" Lee Harvey Oswald for the murder of John F. Kennedy. His book is a narrative compendium of fact, ballistic evidence, and, above all, common sense.
Vocational occupations are attractive not so much for their material rewards as for the prestige and self-fulfillment they confer. They require a strong personal commitment, which can be subjectively experienced in terms of passion and selflessness. The choice of a career in the cultural sector provides a good example of this. What are the terms of this calling? What predisposes individuals to answer it? What are the meanings of such a choice? To answer these questions, this book focuses on would-be cultural managers. By identifying their social patterns, by revealing the resources, expectations and visions of the world they invest in their choice, it sheds new light on these occupations. In these intermediary and indeterminate social positions, family heritages intersect with educational strategies, aspirations of upward mobility with tactics against downward mobility, and social critique with adjustment strategies. Ultimately the study of career choices in cultural management suggests a new take on the analysis of social reproduction and on the embodiment of the new spirit of capitalism. The empirical findings of this research conducted in France are set in a broader comparative perspective, at the European level and with the USA.
Cet ouvrage rassemble les premières nouvelles écrites par l'auteur, entre 1999 et 2003.De "La Féline" à "L'heure du thé", vingt-cinq histoires entre réalisme et merveilleux.
When The Earth, the Temple, and the Gods first appeared in 1962, it was hailed by the critics for it erudition, historical imagination and boldness. Subsequently, this comprehensive study of Greek temples and site-planning has been widely accepted as a landmark of architectural history, for it offers an inspired and arresting insight into nature and function of Greek sacred architecture. Vincent Scully, one of America's most brilliant and articulate scholars, understands the temples as physical embodiment of the gods in landscapes that had for the Greeks divine attributes and sacred connotations. He explores the meanings inherent in the calculated interaction between man-made sculptural forces and the natural landscape, and he relates this interaction to our understanding of Greek culture from the pre-Greek Aegean to the Hellenistic period. Years of research and travel were devoted to The Earth, the Temple, and the Gods. Scores of sites were restudied on the spot, including many lesser-known sanctuaries throughout the Hellenic world. The study includes reconstruction drawings, plans, and maps along with its richly illustrated, detailed discussions of major sites.
André Dupont (1742-1817), is the first rose collector we know of. From 1785 onwards, he developed a passion for this beautiful genus, striving to have a garden where he could produce his Roses and observe them, in Paris, mainly in the surroundings of the Luxembourg Palace. He was not a horticulturist, so he immersed himself in the books lent to him by the National Museum of Natural History, met botanists, erudite men of the Enlightenment, gardeners and nurserymen. He thus became a good connoisseur of the Roses of his time, planning to write a monograph. At the beginning of the 19th century, the fashion for roses began, and André Dupont made a major contribution to this development; he was its forerunner. Madame Bonaparte, Empress Joséphine, brought him great fame, as she turned him into her main supplier of roses to embellish the greenhouses and flowerbeds of her estate in Malmaison. Renowned as the depositary of a complete École de Roses and of the oddities that nature provides them with, he gathered the very first herbarium exclusively devoted to Roses that we may still consult. In 1814, Dupont was at the end of his life and gave his collection to the Luxembourg Gardens. It was the beginning of the largest rose garden in the world that one could admire during most of the 19th century. He could thus observe the flowering of his beloved Roses for two or three more seasons, before passing away not far from them. While his botanist friends, or famous horticulturists, were collecting fame and honours, he ended his life in a boarding house in his neighbourhood, under the threat of a ban and ruined. His Roses, cheerful and good girls, sang his praises for a long time in front of the palace where their gardener had the honour to serve. Very little, too little, has been written about him. The main book of the present work, in French, brings together, for the first time, all the known elements of his life, from the period of the princely court he was a part of, to the 2nd Restoration, through the French Revolution and the 1st Empire. The present booklet proposes, in English, the essential parts of the original text. In the course of this story, André Dupont reveals his temper - sometimes a bit of an eccentric - so imbued with tenderness for his Roses!
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