The Molecular Pathology of Prion Diseases

Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of invariably fatal neurodegenerative disorders. Uniquely, they may present as sporadic, inherited, or infectious forms, all of which involve conversion of the normal cellular prion protein (PrPC) into a pathogenic likeness of itself (PrPSc). Formation of neurotoxic PrPSc and/or loss of the normal function of native PrPC result in activation of cellular pathways ultimately leading to neuronal death. Prion diseases can affect both humans and animals, with scrapie of sheep, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease being the most notable. This review is intended to provide an overview of the salient scientific discoveries in prion research, mainly from a molecular perspective. Further, some of the major outstanding questions in prion science are highlighted. Prion research is having a profound impact on modern medicine, and strategies for prevention and treatment of these disorders may also find application in the more common neurodegenerative diseases.peer-reviewe

The CCD sensor: A semiconductor circuit for capturing images. On the Nobel Prize in Physics awarded to Charles Kuen Kao, Willard S. Boyle, and George E. Smith (II)

The Nobel Prize in Physics 2009 was jointly awarded to two scientists (in addition to Charles Kuen Kao): Willard S. Boyle and George E. Smith, both of Bell Laboratories in Murray Hill, New Jersey, for having invented, in 1969, an imaging semiconductor circuit, the CCD (charge-coupled device), an electronic device which allowed an important development of digital cameras, both photography and video. A year later, Bell Labs already had the first CCD-based camcorder. In commercial cameras it is being replaced by CMOS sensors, but in some capture systems it is still the most important component, especially because of its low levels of noise. This article examines the functioning and evolution of the device.El Premi Nobel de Física 2009 va atorgar el guardó compartit a dos científics (a més de Charles Kuen Kao): Willard S. Boyle i George E. Smith, ambdós dels laboratoris Bell de Murray Hill, a Nova Jersey, per haver inventat, el 1969, un dispositiu electrònic, el sensor CCD (charge-coupled device o ‘dispositiu de càrrega acoblada'), que ha permès un desenvolupament important de les càmeres digitals, tant de fotografia com de vídeo. Només un any més tard, els laboratoris Bell ja tenien en funcionament la primera videocàmera basada en CCD. En càmeres comercials està essent substituït per sensors CMOS, però en certs sistemes de captura encara és el més important, especialment pel baix nivell de soroll. En aquest article s'analitza el funcionament del dispositiu i l'evolució que té

Emergence and Adult Biology of \u3ci\u3eAgrilus Difficilis\u3c/i\u3e (Coleoptera: Buprestidae), a Pest of Honeylocust, \u3ci\u3eGleditsia Triacanthos\u3c/i\u3e

Emergence and adult biology of Agrilus difficilis were examined in relation to its host Gleditsia triacanthos. began as early as 5 June in 1982 and completed as late as 22 July in 1983. Females lived significantly longer, 48 days, than males, 29 days. Average fecundity was one egg per day during a 36-day oviposition period

Pashmina going global: dealing with cultural heritage and authenticity in the Kashmiri shawl business in Mamallapuram, India

This volume engages with a problem that has repeatedly been addressed in the anthropology of globalisation, the more recent anthropology of art and the anthropology of tourism, namely the question of how 'culture and commerce' relate to one another, or what effects tourism and commerce have on the symbolic and social value of material culture and its modes of production. It is based on Anne Herms' MA thesis which was supervised by Prof. Susanne Brandtstädter. The ethnographic theme of this study is the sale of 'real' pashmina shawls by Kashmiri traders in Mamallapuram, a tourist town in the south of India. It is based on empirical research conducted in Mamallapuram between October and December 2018. Kashmiri pashmina shawls have a long history of global circulation, and have been widely regarded as desirable prestige objects. Following the debate on the 'social life of things' initiated by Appadurai, reference is first made to the diverse contexts of meaning between people and things, as well as to the proposition that as a result of commodification and global consumption, cultural artifacts suffer a loss of meaning or authenticity, and object and producer become 'alienated' from one another. The work focuses on three questions: How do traders relate to the shawl? What significance does authenticity - a modern term that arose from the tension between original and copy - have in the local shawl trade and why? How can the sale of Kashmiri shawls be assessed in light of the debate on the commodification of culture? As it turns out, dealers have a close, almost emotional identification with the goods, and take pride in their sale and worldwide distribution. Their personal relationship with these objects seems to embody a special 'sociality' of handcrafted shawls and an idea of pashmina as a Kashmiri cultural heritage. Authenticity is of major importance in the (local) shawl business, for tourists and dealers alike. Herms describes how pashmina shawls that come to Mamallapuram as commodities receive (back) the 'aura' of the authentic, and argues that the production of authenticity not only increases the economic value of the goods, but also their symbolic and social meaning. The concepts of 'commodity', 'art' and 'cultural heritage' are practically not separate categories here. The traders' cultural appreciation of the shawl is also due to its commodification, its marketing history and the continuous sale on site. Thus, traders do not become alienated from the product, but rather connected to it

An apocalypse for the Church and for the world : a literary-narrative and tradition-historical reading of the Book of Revelation

EThOS - Electronic Theses Online ServiceGBUnited Kingdo

Monoacylated Cellular Prion Proteins Reduce Amyloid-beta-Induced Activation of Cytoplasmic Phospholipase A2 and Synapse Damage

Alzheimer’s disease (AD) is a progressive neurodegenerative disease characterized by the accumulation of amyloid-β (Aβ) and the loss of synapses. Aggregation of the cellular prion protein (PrPC) by Aβ oligomers induced synapse damage in cultured neurons. PrPC is attached to membranes via a glycosylphosphatidylinositol (GPI) anchor, the composition of which affects protein targeting and cell signaling. Monoacylated PrPC incorporated into neurons bound “natural Aβ”, sequestering Aβ outside lipid rafts and preventing its accumulation at synapses. The presence of monoacylated PrPC reduced the Aβ-induced activation of cytoplasmic phospholipase A2 (cPLA2) and Aβ-induced synapse damage. This protective effect was stimulus specific, as treated neurons remained sensitive to α-synuclein, a protein associated with synapse damage in Parkinson’s disease. In synaptosomes, the aggregation of PrPC by Aβ oligomers triggered the formation of a signaling complex containing the cPLA2.a process, disrupted by monoacylated PrPC. We propose that monoacylated PrPC acts as a molecular sponge, binding Aβ oligomers at the neuronal perikarya without activating cPLA2 or triggering synapse damage

Saving a Tree Genus: Researchers Are Working Toward an Ash Hybrid Resistant to Emerald Ash Borer

Author Institution (Herms): Department of Entomology, The Ohio State University; Author Institution (Bonello): Department of Plant Pathology, The Ohio State Universit