2,095 research outputs found

    A murine model of variant late infantile ceroid lipofuscinosis recapitulates behavioral and pathological phenotypes of human disease.

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    Neuronal ceroid lipofuscinoses (NCLs; also known collectively as Batten Disease) are a family of autosomal recessive lysosomal storage disorders. Mutations in as many as 13 genes give rise to ∼10 variants of NCL, all with overlapping clinical symptomatology including visual impairment, motor and cognitive dysfunction, seizures, and premature death. Mutations in CLN6 result in both a variant late infantile onset neuronal ceroid lipofuscinosis (vLINCL) as well as an adult-onset form of the disease called Type A Kufs. CLN6 is a non-glycosylated membrane protein of unknown function localized to the endoplasmic reticulum (ER). In this study, we perform a detailed characterization of a naturally occurring Cln6 mutant (Cln6(nclf)) mouse line to validate its utility for translational research. We demonstrate that this Cln6(nclf) mutation leads to deficits in motor coordination, vision, memory, and learning. Pathologically, we demonstrate loss of neurons within specific subregions and lamina of the cortex that correlate to behavioral phenotypes. As in other NCL models, this model displays selective loss of GABAergic interneuron sub-populations in the cortex and the hippocampus with profound, early-onset glial activation. Finally, we demonstrate a novel deficit in memory and learning, including a dramatic reduction in dendritic spine density in the cerebral cortex, which suggests a reduction in synaptic strength following disruption in CLN6. Together, these findings highlight the behavioral and pathological similarities between the Cln6(nclf) mouse model and human NCL patients, validating this model as a reliable format for screening potential therapeutics

    Evaluating Hunting Success of Pen-Reared and Wild Northern Bobwhite in a Reclaimed Kentucky Mineland

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    Northern bobwhites (Colinus virginianus) have experienced severe population declines across their distribution. In order to address population declines and to continue providing hunting opportunities, multi-state efforts have been undertaken to stabilize and restore bobwhite populations. Ongoing efforts using the National Bobwhite Conservation Initiative’s quail focus area approach have so far demonstrated success throughout Kentucky. However, population increases in the Peabody Bobwhite Focal Area, in western Kentucky, have not been correlated to increases in perceived hunter success. Consequently, some sportsmen question the effectiveness of focal area conservation. In response to hunter concerns, we tested dog hunting ability with wild and pen-reared bobwhites. We also measured evasive behaviors of wild bobwhite using radiotelemetry. During the 2013–2014 and 2014–2015 hunting seasons we conducted 114 dog trials. Dogs detected bobwhite during 46 of 59 (78.0%) pen-reared trials and 16 of 55 (29.1%) wild bird trials. When dogs did not detect wild quail, birds ran away 64.1% of the time and remained motionless 20.5% of the time. Using an information-theoretic approach, we determined that bird type (wild vs. pen-reared) had a significant effect on bird detection, with dogs 8.62 times more likely to detect pen-reared birds than wild birds. We recommend that hunters be informed about differences in dog detection rates between pen-reared and wild bobwhite so that public support needed for wild bobwhite restoration can persist

    Temperature Assessment on a Reclaimed Surface Mine During Northern Bobwhite Breeding Season: Considerations for Habitat Management

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    Ground-level air temperatures were assessed within 4 distinct habitat areas on a managed reclaimed surface mine at Peabody Wildlife Management Area, Kentucky, 26 June–17 July 2015, during the northern bobwhite (Colinus virginianus) brood season. Habitat consisted of disked and nondisked areas of native grass and an invasive species, sericea lespedeza (Lespedeza cuneata). Disked areas offered more open space for bobwhite mobility and experienced higher average temperatures than nondisked sites. Although statistically significant, differences in air temperature between disked and nondisked areas were likely too small to have practical implications for bobwhite habitat management in Kentucky under current climatic conditions. This will likely change in the future as the regional climate warms and periods of excessive heat are more likely to occur. Consequently, managers may want to consider microclimate when making management decisions

    Wildfire risk for main vegetation units in a biodiversity hotspot : modeling approach in New Caledonia, South Pacific

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    Wildfire has been recognized as one of the most ubiquitous disturbance agents to impact on natural environments. In this study, our main objective was to propose a modeling approach to investigate the potential impact of wildfire on biodiversity. The method is illustrated with an application example in New Caledonia where conservation and sustainable biodiversity management represent an important challenge. Firstly, a biodiversity loss index, including the diversity and the vulnerability indexes, was calculated for every vegetation unit in New Caledonia and mapped according to its distribution over the New Caledonian mainland. Then, based on spatially explicit fire behavior simulations (using the FLAMMAP software) and fire ignition probabilities, two original fire risk assessment approaches were proposed: a one-off event model and a multi-event burn probability model. The spatial distribution of fire risk across New Caledonia was similar for both indices with very small localized spots having high risk. The patterns relating to highest risk are all located around the remaining sclerophyll forest fragments and are representing 0.012% of the mainland surface. A small part of maquis and areas adjacent to dense humid forest on ultramafic substrates should also be monitored. Vegetation interfaces between secondary and primary units displayed high risk and should represent priority zones for fire effects mitigation. Low fire ignition probability in anthropogenic-free areas decreases drastically the risk. A one-off event associated risk allowed localizing of the most likely ignition areas with potential for extensive damage. Emergency actions could aim limiting specific fire spread known to have high impact or consist of on targeting high risk areas to limit one-off fire ignitions. Spatially explicit information on burning probability is necessary for setting strategic fire and fuel management planning. Both risk indices provide clues to preserve New Caledonia hot spot of biodiversity facing wildfires

    Morally Respectful Listening and its Epistemic Consequences

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    What does it mean to listen to someone respectfully, that is, insofar as they are due recognition respect? This paper addresses that question and gives the following answer: it is to listen in such a way that you are open to being surprised. A specific interpretation of this openness to surprise is then defended

    Reconfigurable and Tunable Metamaterials: A Review of the Theory and Applications

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    Metamaterials are being applied to the development and construction of many new devices throughout the electromagnetic spectrum. Limitations posed by the metamaterial operational bandwidth and losses can be effectively mitigated through the incorporation of tunable elements into the metamaterial devices. There are a wide range of approaches that have been advanced in the literature for adding reconfiguration to metamaterial devices all the way from the RF through the optical regimes, but some techniques are useful only for certain wavelength bands. A range of tuning techniques span from active circuit elements introduced into the resonant conductive metamaterial geometries to constituent materials that change electromagnetic properties under specific environmental stimuli. This paper presents a survey of the development of reconfigurable and tunable metamaterial technology as well as of the applications where such capabilities are valuable

    The institutional shaping of management: in the tracks of English individualism

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    Globalisation raises important questions about the shaping of economic action by cultural factors. This article explores the formation of what is seen by some as a prime influence on the formation of British management: individualism. Drawing on a range of historical sources, it argues for a comparative approach. In this case, the primary comparison drawn is between England and Scotland. The contention is that there is a systemic approach to authority in Scotland that can be contrasted to a personal approach in England. An examination of the careers of a number of Scottish pioneers of management suggests the roots of this systemic approach in practices of church governance. Ultimately this systemic approach was to take a secondary role to the personal approach engendered by institutions like the universities of Oxford and Cambridge, but it found more success in the different institutional context of the USA. The complexities of dealing with historical evidence are stressed, as is the value of taking a comparative approach. In this case this indicates a need to take religious practice as seriously as religious belief as a source of transferable practice. The article suggests that management should not be seen as a simple response to economic imperatives, but as shaped by the social and cultural context from which it emerges
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