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Selected AbstractsAnimal models of Williams syndrome,AMERICAN JOURNAL OF MEDICAL GENETICS, Issue 2 2010Lucy R. Osborne Abstract In recent years, researchers have generated a variety of mouse models in an attempt to dissect the contribution of individual genes to the complex phenotype associated with Williams syndrome (WS). The mouse genome is easily manipulated to produce animals that are copies of humans with genetic conditions, be it with null mutations, hypomorphic mutations, point mutations, or even large deletions encompassing many genes. The existing mouse models certainly seem to implicate hemizygosity for ELN, BAZ1B, CLIP2, and GTF2IRD1 in WS, and new mice with large deletions of the WS region are helping us to understand both the additive and potential combinatorial effects of hemizygosity for specific genes. However, not all genes that are haploinsufficient in humans prove to be so in mice and the effect of genetic background can also have a significant effect on the penetrance of many phenotypes. Thus although mouse models are powerful tools, the information garnered from their study must be carefully interpreted. Nevertheless, mouse models look set to provide a wealth of information about the neuroanatomy, neurophysiology and molecular pathways that underlie WS and in the future will act as essential tools for the development and testing of therapeutics. © 2010 Wiley-Liss, Inc. [source] Trace Element Determination by ICP-AES and ICP-MS: Developments and Applications Reported During 2004 and 2005GEOSTANDARDS & GEOANALYTICAL RESEARCH, Issue 3 2006Kathryn L. Linge This review describes significant developments in trace element determination using inductively coupled plasma-atomic emission spectrometry (ICP-AES) and inductively coupled plasma-mass spectrometry (ICP-MS) that were reported in 2004 and 2005. It focuses on the application of ICP techniques to geological and environmental samples; fundamental studies in ICP-MS and ICP-AES instrumentation are not included. The literature reviewed indicated that the majority of new publications concerned advances in ICP-MS analysis rather than ICP-AES. However, ICP-AES developments are still being published, particularly in the areas of sample preconcentration and sample introduction. The trend in increasing publication of developments in hyphenated speciation techniques looks set to persist as knowledge of elemental speciation becomes critical for many environmental studies. Collision or reactions cells were the most reported technique for spectral interference removal in ICP-MS, probably reflecting the growing adoption of cell instruments in laboratories during the last few years. [source] Missile defence and the transatlantic security relationshipINTERNATIONAL AFFAIRS, Issue 3 2001Wyn Q. Bowen This article examines the re-emergence of ballistic missile defence (BMD) as a contentious issue in US-European security relations since 1999. It begins by outlining three phases in the recent evolution of US missile defence policy from 1995 to mid-2001. The article then examines five key factors that have dominated European views and concerns in relation to BMD: a divergence between European and American assessments of the emerging ballistic missile threat; concern over the implications for nuclear arms control stemming from Russian and Chinese opposition to BMD; the impact of missile defence on deterrence and the Atlantic alliance; scepticism about the technological feasibility of BMD; and the potential opportunity costs associated with resource allocation to missile defence. It is shown that European anxieties have been exacerbated by a perception of a growing unilateralism in American security policy in recent years. The article proceeds by arguing that the US-European debate over BMD looks set to evolve in one of two directions. The more likely and most desirable scenario would involve the US reaching an understanding with its European allies on the way forward. The less desirable scenario would involve key European countries, such as France and Germany, deciding ultimately to withhold their political support for BMD, which would have the potential of causing significant rifts in both transatlantic and intra-European security relations. In both cases, it is argued that the BMD debate will be defined by the interaction of several key variables. These include the extent to which the Bush administration engages in meaningful consultations with the Europeans; the administration's ability or otherwise to reach an agreement with Russia on the way ahead; the architecture options of a future allied or global BMD system; the related issues of technological feasibility and financial cost; and the evolving missile threat. [source] Middle East set to benefit as petrochemical demand growsOIL AND ENERGY TRENDS, Issue 8 2005Article first published online: 15 AUG 200 Strong worldwide demand for petrochemicals, particularly in China, is prompting many oil and petrochemical producers to build new ethylene capacity. China itself has extensive plans for capacity additions, as do some other Asian countries, but the largest increase looks set to come from the Middle East. Here, the growth will be based on access to abundant low-cost feedstocks, especially gas, which will give the region a considerable advantage over regions dependent on expensive oil-based feedstocks, including much of Asia. The exploitation of gas-based feedstocks has enabled the Middle East to build up a significant presence in world ethylene markets already. Since 2000, it has been the largest exporter of ethylene and its dominance could grow considerably over the coming decade, largely at the expense of the US. On present projections of demand and capacity, the Middle East is likely to account for well over 90% of the world's net trade in ethylene by 2010, with China as its principal market. [source] Ion-dependent gating of kainate receptorsTHE JOURNAL OF PHYSIOLOGY, Issue 1 2010Derek Bowie Ligand-gated ion channels are an important class of signalling protein that depend on small chemical neurotransmitters such as acetylcholine, l -glutamate, glycine and ,-aminobutyrate for activation. Although numerous in number, neurotransmitter substances have always been thought to drive the receptor complex into the open state in much the same way and not rely substantially on other factors. However, recent work on kainate-type (KAR) ionotropic glutamate receptors (iGluRs) has identified an exception to this rule. Here, the activation process fails to occur unless external monovalent anions and cations are present. This absolute requirement of ions singles out KARs from all other ligand-gated ion channels, including closely related AMPA- and NMDA-type iGluR family members. The uniqueness of ion-dependent gating has earmarked this feature of KARs as a putative target for the development of selective ligands; a prospect all the more compelling with the recent elucidation of distinct anion and cation binding pockets. Despite these advances, much remains to be resolved. For example, it is still not clear how ion effects on KARs impacts glutamatergic transmission. I conclude by speculating that further analysis of ion-dependent gating may provide clues into how functionally diverse iGluRs families emerged by evolution. Consequently, ion-dependent gating of KARs looks set to continue to be a subject of topical inquiry well into the future. [source] |