Williams Syndrome is a developmental disorder that is characterized by cardiovascular problems, particular facial features and several typical behavioral and neurological abnormalities. In Williams Syndrome patients, a heterozygous deletion is present of a region on chromosome 7q11.23 (the Williams Syndrome critical region), which spans approximately 20 genes. Two of these genes encode proteins that regulate dynamic aspects of the cytoskeleton of the cell, either via the actin filament system (LIM kinase 1, or LIMK1), or through the microtubule network (cytoplasmic linker (...) protein of 115 kDa, or CLIP‐115). The recent findings that knockout mice lacking LIMK1 or CLIP‐115 have distinct neurological and behavioural phenotypes, indicates that cytoskeletal defects might play a role in the development of neurological symptoms in Williams Syndrome patients. In this review, we discuss the properties of LIMK and CLIP family proteins, their function in the regulation of the actin and microtubule cytoskeletal systems, respectively, and the relationship with neurodevelopmental aspects of Williams Syndrome. BioEssays 26:141–150, 2004. © 2004 Wiley Periodicals, Inc. (shrink)

Several themes can be identified in the commentaries. The first is that the climbing fibers may have more than one function; the second is that the climbing fibers provide sensory rather than motor signals. We accept the possibility that climbing fibers may have more than one function consequence(s)’ in the title. Until we know more about the function of the inhibitory input to the inferior olive from the cerebellar nuclei, which are motor structures, we have to keep open the possibility (...) that the climbing fiber signals can be a combination of sensory and motor signals. (shrink)

Despite its uniform appearance, the cerebellar cortex is highly heterogeneous in terms of structure, genetics and physiology. Purkinje cells (PCs), the principal and sole output neurons of the cerebellar cortex, can be categorized into multiple populations that differentially express molecular markers and display distinctive physiological features. Such features include action potential rate, but also their propensity for synaptic and intrinsic plasticity. However, the precise molecular and genetic factors that correlate with the differential physiological properties of PCs remain elusive. In this (...) article, we provide a detailed overview of the cellular mechanisms that regulate PC activity and plasticity. We further perform a pathway analysis to highlight how molecular characteristics of specific PC populations may influence their physiology and plasticity mechanisms. (shrink)

Background Reasonable disagreement about the role awarded to gamete donors in decision-making on the use of embryos created by gamete donation for research purposes emphasises the importance of considering the implementation of participatory, adaptive, and trustworthy policies and guidelines for consent procedures. However, the perspectives of gamete donors and recipients about decision-making regarding research with EGDs are still under-researched, which precludes the development of policies and guidelines informed by evidence. This study seeks to explore the views of donors and recipients (...) about who should take part in consent processes for the use of EGDs in research. Methods From July 2017 to June 2018, 72 gamete donors and 175 recipients completed a self-report structured questionnaire at the Portuguese Public Bank of Gametes. Agreement with dual consent was defined as the belief that the use of EGDs in research should be consented by both donors and recipients. Results The majority of participants were willing to donate embryos for research. Almost half of the donors and half of the recipients considered that a dual consent procedure is desirable. This view was more frequent among employed recipients than among non-employed. Donors were less likely to believe that only recipients should be involved in giving consent for the use of EGDs in research and were more frequently favourable to the idea of exclusive donors’ consent. Conclusions Divergent views on dual consent among donors and recipients indicate the need to develop evidence-based and ethically sustainable policies and guidelines to protect well-being, autonomy and reproductive rights of both stakeholder groups. More empirical research and further theoretical normative analyses are needed to inform people-centred policy and guidelines for shared decision-making concerning the use of EGDs for research. (shrink)

The search for knowledge has continued to expand to new domains since its start in the seventeenth century. Some of them have proved unusually resistant. Methods have had to proliferate to deal with the obstacles, for example in the social domain. There also have been ideological reactions. Surprisingly frequently, methods and activities that appear to be effective in dealing with such domains are classified as "preliminary" or are distinguished by a "point of view" that has yet to be transcended to (...) achieve "true" knowledge. One such activity is the acquisition of high quality experience. It is argued in the paper that it does not deserve being treated as a poor relative. It has a history of its own and can point to many successes, for example in the development of new values and emotions. Its only drawback seems to be that the search for high quality experiences has tended to be heuristic, or if one wishes, artistic. This situation is changing, however. In the paper the differences between the acquisition of knowledge and that of high quality experience are delineated. It is argued that facilitation of the latter's searches requires the development of interactions between entities that generate and structure experiences--i.e., of human collectives that stabilise sufficiently to execute a collective action in their environment. They are characterised by the use of coordinating languages. (shrink)

Prison work creates ethical challenges for which a training program was initiated for Dutch prison staff to foster their Moral Craftsmanship (MCS). The concept of MCS is not yet defined and operationalized in literature. This explorative study aims to 1) define MCS, 2) identify conceptual elements of MCS, and 3) develop a measurement tool for MCS. A document and literature study provided input for the definition and selection of conceptual elements related within DCIA policy documents, identifying three conceptual levels of (...) MCS: individual (cognition, attitude, and actions), team, and leadership. This first MCSQ consists of seventy-one items distributed across the domains: values and norms, judgment and reasoning, consultation, implementation, looking back, interactions, and leadership. We tested its usability via ‘think-aloud interviews.’ Validation of the current version of the MCS-Questionnaire and consensus among experts about what to include or exclude in the concept of MCS are needed for further development. (shrink)

Evolutionary developmental biology (evo-devo) has undergone dramatic transformations since its emergence as a distinct discipline. This paper aims to highlight the scope, power, and future promise of evo-devo to transform and unify diverse aspects of biology. We articulate key questions at the core of eleven biological disciplines—from Evolution, Development, Paleontology, and Neurobiology to Cellular and Molecular Biology, Quantitative Genetics, Human Diseases, Ecology, Agriculture and Science Education, and lastly, Evolutionary Developmental Biology itself—and discuss why evo-devo is uniquely situated to substantially improve (...) our ability to find meaningful answers to these fundamental questions. We posit that the tools, concepts, and ways of thinking developed by evo-devo have profound potential to advance, integrate, and unify biological sciences as well as inform policy decisions and illuminate science education. We look to the next generation of evolutionary developmental biologists to help shape this process as we confront the scientific challenges of the 21st century. (shrink)

'This book is an excellent, theoretically sound and politically relevant reader', Professor Wolfschaefer, Universitat des Bundeswehr, Hamburg 'Up to date complete overview of European monetary and fiscal policy issues. Highly readable, good mix of theory and data' 'I think the book contains a wealth of useful, precise information, presented in a straightforward, readable way in a quintessentially comparative perspective', Dr M Mclean, Royal Holloway University 'Excellent treatment - quite comprehensive, full references, accessible for non-economists', Charlotte Bretherton, Liverpool John Moores Univesity (...) 'A useful addition to student literature', MJ Macmillan, Exeter University 'This is a top class book and does not limit itself to assessing the adequacy of the European political economy to the precepts of advanced monetary economics...This book is an excellent tool for teaching EMU economics, and raising the level of EMU economics in the reaserch-field of international political economy. Students and scholars will appreciate the explicit assessment the authors make of complex economic issues, and their unbiased stance in the study of EMU economic and political interplay.' Miriam L.Campamella, Jean Monnet Professor, Faculty of Political Science, University of Turin. ECSA Review, Winter 2001This textbook offers a fresh and comprehensive examination of European monetary and fiscal policy in the third stage of Economic and Monetary Union. Professors Eijffinger and de Haan give a brief history of European economic integration before the transition to EMU, and continue with a comprehensive analysis of institutions, legislation, and policies. Their analysis includes the functions and goals of the European Central Bank, the Treaty on European Union, the Stability and Growth Pact, and the harmonization of taxes. Other topics discussed include the targets and instruments of European monetary policy, the integration of European financial markets, and the competition between financial institutions in Europe. (shrink)

This paper, which gives continuity to another article, analyzes the content of I Dialogus V, 14-22 and, in an appendix, presents our Portuguese translation of this excerpt. In these chapters, the Inceptor Venerabilis discusses whether St. Peter and the Roman Church possess primacy over all other apostles and churches; andwhether this primacy is granted by God himself. Ockham first presents, not ad litteram, the opinion of those who refute the thesis that Christ did not give primacy to Peter and the (...) Roman Church. Then, because this thesis fully deflates papal power, he proposes a contrary thesis based on five proofs. We conclude that Ockham’s unnamed opponent is Marsilius of Padua, whose opinions are in his Defensor Pacis. (shrink)

The US neutrino community gathered at the Workshop on the Intermediate Neutrino Program at Brookhaven National Laboratory February 4-6, 2015 to explore opportunities in neutrino physics over the next five to ten years. Scientists from particle, astroparticle and nuclear physics participated in the workshop. The workshop examined promising opportunities for neutrino physics in the intermediate term, including possible new small to mid-scale experiments, US contributions to large experiments, upgrades to existing experiments, R&D plans and theory. The workshop was organized into (...) two sets of parallel working group sessions, divided by physics topics and technology. Physics working groups covered topics on Sterile Neutrinos, Neutrino Mixing, Neutrino Interactions, Neutrino Properties and Astrophysical Neutrinos. Technology sessions were organized into Theory, Short-Baseline Accelerator Neutrinos, Reactor Neutrinos, Detector R&D and Source, Cyclotron and Meson Decay at Rest sessions.This report summarizes discussion and conclusions from the workshop. (shrink)

Human rights advocates often use inflated and thus underspecified terminologies when addressing the content of their claims. One example of such loose terminology is the term ‘well-being’, as currently employed in connection with a definition for the right to health. What I call the ‘well-being conception of health’ conflates the distinct ideas of basic and non-basic health needs, as well as those of individual autonomy and freedom. I call this the conflation problem. This paper argues for the need of an (...) understanding of the right to health, nuanced enough to capture not only these distinct ideas, but also their moral relevance for the common good. (shrink)

Computer vision research involving street and road detection methods usually focuses on driving assistance and autonomous vehicle systems. In this context, street segmentation occurs in real-time, based on images centered on the street. This work, on the other hand, uses street segmentation for urban planning research to classify pavement types of a city or region, which is particularly important for developing countries. For this application, it is needed a dataset with images from various locations for each street. These images are (...) not necessarily centered on the street and include challenges that are not common in street segmentation datasets, such as mixed pavement types and the presence of faults and holes on the street. We implemented a multi-class version of a state-of-the-art segmentation algorithm and adapted it to perform street pavement classification, handling navigation along streets and angle variation to increase the accuracy of the classification. A data augmentation approach is also proposed to use preliminary results from the test instances as new ground truth and increase the amount of training data. A dataset with more than 300,000 images from 773 streets from a Brazilian city was built. Our approach achieved a precision of 0.93, showing the feasibility of the proposed application. (shrink)

© 2016 Regents of the University of California.The Review summarizes much of particle physics and cosmology. Using data from previous editions, plus 3,062 new measurements from 721 papers, we list, evaluate, and average measured properties of gauge bosons and the recently discovered Higgs boson, leptons, quarks, mesons, and baryons. We summarize searches for hypothetical particles such as supersymmetric particles, heavy bosons, axions, dark photons, etc. All the particle properties and search limits are listed in Summary Tables. We also give numerous (...) tables, figures, formulae, and reviews of topics such as Higgs Boson Physics, Supersymmetry, Grand Unified Theories, Neutrino Mixing, Dark Energy, Dark Matter, Cosmology, Particle Detectors, Colliders, Probability and Statistics. Among the 117 reviews are many that are new or heavily revised, including those on Pentaquarks and Inflation. The complete Review is published online in a journal and on the website of the Particle Data Group. The printed PDG Book contains the Summary Tables and all review articles but no longer includes the detailed tables from the Particle Listings. A Booklet with the Summary Tables and abbreviated versions of some of the review articles is also available. (shrink)