X-linked dominant Conradi-Hunermann syndrome is one of a group of disorders with aberrant punctate calcification in cartilage, or chondrodysplasia punctata. This is most prominent around the vertebral column, pelvis and long bones in CPDX2. Additionally, CDPX2 patients may have asymmetric rhizomesomelia, sectorial cataracts, patchy alopecia, ichthyosis and atrophoderma. The phenotype in CDPX2 females ranges from stillborn to mildly affected individuals identified in adulthood. CDPX2 is presumed lethal in males, although a few affected males have been reported. We found increased 8-cholestenol (...) and 8-dehydrocholesterol in tissue samples from seven female probands with CDPX2. This pattern of accumulated cholesterol intermediates suggested a deficiency of 3β-hydroxysteroid- Δ8,Δ7-isomerase, which catalyses an intermediate step in the conversion of lanosterol to cholesterol. A candidate gene encoding a sterol-Δ8-isomerase has been identified and mapped to Xp11.22-p11.23. Using SSCP analysis and sequencing of genomic DNA, we found EBP mutations in all probands. We confirmed the functional significance of two missense alleles by expressing them in a sterol-Δ8- isomerase-deficient yeast strain. Our results indicate that defects in sterol-Δ8-isomerase cause CDPX2 and suggest a role for sterols in bone development. (shrink)

Until today the importance of Marcel Mauss for the emergence andfurther development of Durkheimian sociology of religion has been neglectedboth in religious studies and the sociology of religion. Mauss’ works are, however,more than preliminary steps for Durkheim’s The Elementary Forms of ReligiousLife. This can already be seen in his first major review from 1896 which developsthe concept of the sacred. In general, Mauss’ studies on the sacred and on magic,his analyses of the seasonal variation of religion and of sacrifice and (...) prayer aswell as his conceptualization of mana open new approaches and insights forcurrent studies of religion which go beyond Durkheim’s sociology of religion. Thearticle discusses Mauss’ pioneering role for the Durkheimian perspective onreligion and introduces the central concepts of Mauss’ sociology of religion. (shrink)

Das Jahrbuch für Theorie und Geschichte der Soziologie trägt dem Umstand Rechnung, dass die Soziologie eine Wissenschaft ist, die es mit einem sich historisch wandelnden Gegenstand zu tun hat, also eine Wissenschaft ist, die sich stets von Neuem selbst reflektieren muss, und widmet sich der engen Verbindung von soziologischer Theorie- und Disziplingeschichte sowie allgemein der Reflexionsgeschichte der Gesellschaft und ihren verschiedenen Selbstbeschreibungen. Neben Aufsätzen zur Theorie und Geschichte der Soziologie enthalten die einzelnen Bände dieses Jahrbuches auch Nachrichten aus der soziologiegeschichtlichen (...) Forschung, Inedita aus den verschiedenen einschlägigen Archiven, Interviews mit Zeitzeugen sowie Besprechungen einschlägiger Buchpublikationen zu diesem Thema. Der Inhalt Editorial • Aufsätze • Diskussion • Nachrichten aus der soziologiegeschichtlichen Forschung • Editionsprojekte • Unveröffentlichtes aus den Archiven • Rezensionen • Anhang Die Zielgruppe SoziologInnen Die Herausgeber Dr. Martin Endreß ist Professor für Allgemeine Soziologie an der Universität Trier. Dr. Stephan Moebius ist Professor für Soziologische Theorie und Ideengeschichte an der Karl-Franzens-Universität Graz. (shrink)

This chapter takes a deeper look into the impact of cooperative approaches within modern societies, especially of the transformation into resilient responsible societies. Therefore, we discuss the background and the development of resilience at individual, organizational, and societal levels. Especially the societal resilience needs to be more specified and examined. We place as a central assumption that every citizen is a co-producer of resilience in the state and in society. The cooperative approach can deliver many impulses needed. We are taking (...) a look at the similarities and differences between cooperatives and all forms of resilient organizations. (shrink)

This is the first comprehensive translation of the expanded French version of E.F.F. Chladni's Traité d'Acoustique, using Chladni's 1802 Die Akustik for reference and clarification. Chladni's experiments and observations with sound and vibrations profoundly influenced the development of the field of Acoustics. The famous Chladni diagrams along with other observations are contained in Die Akustik, published in German in 1802 and Traité d'Acoustique, a greatly expanded version, published in French in 1809. The present translation was undertaken by Robert T. Beyer, (...) PhD (1920-2008), noted acoustician, Professor of Physics at Brown University, and Gold Medal recipient of the Acoustical Society of America. Along with many other projects completed over the course of his career, Dr. Beyer translated Von Neumann's seminal work, Mathematical Foundations of Quantum Mechanics from the original German, spent 30 years translating Russian physics treatises and journals, served as editor of the English translation of the Soviet Journal of Experimental and Theoretical Physics, and also authored Sounds of our Times: Two Hundred Years of Acoustics. Represents the first comprehensive English translation of a seminal work in acoustics Translated by a prominent acoustician, and former Gold Medal recipient of the Acoustical Society of America In addition to the classic experiments with glass plates and violin bows, covers topics in music, architecture, and anatomy of human and animal hearing Appeals to a wide audience in physics, acoustics, and history of science. (shrink)

From the time of the thinkers of ancient Greece, the question of what can be said about the existence and nature of God has been debated by many philosophers and theologians. In Theism and Atheism: The Eternal Debate, F.F. Centore presents a broad analysis of the major positions that address the question and the thinkers who have contributed to the debate. This is an admirably lucid and thorough examination of the history of natural theology. Covering the material in a thematic (...) rather than in a strictly chronological way, Centore draws out the key positions on topics such as the relationship of science and religion, the problem of evil, Naturalistic and Supernaturalistic theism and the dispute between essence and existence. The proponents of the various positions are considered alongside their most explicit critics. Important thinkers dealt with in this work include Plato, Aristotle, Aquinas, Descartes, Pascal, Kant, Hume, Hegel, Nietzsche, Freud and Darwin. The diversity of exploration also goes beyond the Judeo-Christian tradition to look at Hinduism, Buddhism, Stoicism, Zoroastrianism, Confucianism and Classical Paganism. This work, however, is much more than a survey of the major historical arguments and rebuttals concerning God's existence and nature. Overall, it is a work by a well-established Catholic philosopher who concludes that God's existence and nature are indeed compatible with modern science, human freedom and the existence of evil in the world. (shrink)

The adventurous hero Indiana Jones makes his long-awaited return to theaters this summer! Filled with the intrigue and drama characteristic of all the classic Indiana Jones films, the fourth release is all brought to life by an incredible soundtrack. Selections from Indiana Jones and the Kingdom of the Crystal Skull features popular music from the film, as composed by Academy Award winner John Williams, plus 8 pages of color artwork straight from the movie. This book is part of an instrumental (...) series arranged for Flute, Clarinet, Alto Sax, Tenor Sax, Trumpet, Horn in F, and Trombone. The arrangements are completely compatible with each other and can be played together as solos. Each book contains a carefully edited part that is appropriate for the Level 2–3 player, and a fully orchestrated accompaniment CD. The CD includes a demo track of each song, which features a live instrumental performance, followed by a play-along track. A piano accompaniment book (optional) is also available. It includes a CD that features various instrument demo tracks from the series. Titles include: *Raiders March* *The Adventures of Mutt* *The Journey to Akator* *Marion’s Theme* *Irina’s Theme*. (shrink)

For Paul and Luke alike, the present age is the age of the Spirit; for them both, the presence and activity of the Spirit constituted the great new fact of their time.

CCP is not, generally speaking, well-respected today. This is not so much because CCP is impure, being, as it is, all mixed up with dogmatic statements drawn from Scripture, Church teachings, and so on, but because CCP is impure in a certain way. All philosophies are always and everywhere impure in the sense of always and everywhere being embedded in some particular time and culture. Philosophies are written by philosophers, each of whom is taught many things at his mother’s knee (...) long before he ever becomes a philosopher. What he is taught, though, will vary, and some doctrines, it would seem, are more damaging than others to what today is considered good philosophizing. My purpose in this paper is to set out the obnoxious form of impurity which is generally thought to invalidate CCP, and then briefly to provide some hints as to why the situation is not at all as bad as the critics make it out to be. (shrink)

THE aim of this paper is to examine the reaction of an Aristotelian and a Cartesian to Newton’s 1672 paper on light and colors. The Aristotelian is Francis Hall, an English Jesuit who held the position of professor of mathematics in the Jesuit college at Liège. In his own time he was better known by his pseudonym Francis Line or Linus. The Cartesian is Robert Hooke, the Curator of Experiments to the Royal Society of London since its official beginning in (...) 1662. It is not my purpose to pass judgment on Newton’s theory itself or to analyze his replies. Rather it is to investigate the ways in which two intellectual attitudes, representing two different carry-over philosophical orientations, approached the same problem. I shall set out Newton’s main thesis, the criticisms directed against it by Hooke and Linus and then evaluate the merits and demerits of each approach. (shrink)

This work is a paperback reissue, with an added preface, of a 1959 anthology of essays on the background to Darwin’s Origin of Species. It was meant to serve two purposes. One was to honor Darwin on the hundredth anniversary of his famous book. The other was to honor Professor A O Lovejoy, the Johns Hopkins scholar who did so much to popularize process philosophy in the United States. About half of the new preface is devoted to an extensive, though (...) by no means complete, bibliographic note on recent Darwinian scholarship. Over-all this is an excellent volume. It does, however, have certain shortcomings. (shrink)

“… the recipients of this letter had to learn that while Christ is unchanging, he is nevertheless onward-moving, always leading his people forth to new ventures in his cause…. He is the pioneer, the trailblazer, along the path of faith….”.