1,986 research outputs found

    Poxviruses in bats … so what?

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    Poxviruses are important pathogens of man and numerous domestic and wild animal species. Cross species (including zoonotic) poxvirus infections can have drastic consequences for the recipient host. Bats are a diverse order of mammals known to carry lethal viral zoonoses such as Rabies, Hendra, Nipah, and SARS. Consequent targeted research is revealing bats to be infected with a rich diversity of novel viruses. Poxviruses were recently identified in bats and the settings in which they were found were dramatically different. Here, we review the natural history of poxviruses in bats and highlight the relationship of the viruses to each other and their context in the Poxviridae family. In addition to considering the zoonotic potential of these viruses, we reflect on the broader implications of these findings. Specifically, the potential to explore and exploit this newfound relationship to study coevolution and cross species transmission together with fundamental aspects of poxvirus host tropism as well as bat virology and immunology

    When signalling goes wrong:pathogenic variants in structural and signalling proteins causing cardiomyopathies

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    Cardiomyopathies are a diverse group of cardiac disorders with distinct phenotypes, depending on the proteins and pathways affected. A substantial proportion of cardiomyopathies are inherited and those will be the focus of this review article. With the wide application of high-throughput sequencing in the practice of clinical genetics, the roles of novel genes in cardiomyopathies are recognised. Here, we focus on a subgroup of cardiomyopathy genes [TTN, FHL1, CSRP3, FLNC and PLN, coding for Titin, Four and a Half LIM domain 1, Muscle LIM Protein, Filamin C and Phospholamban, respectively], which, despite their diverse biological functions, all have important signalling functions in the heart, suggesting that disturbances in signalling networks can contribute to cardiomyopathies

    Let your maps be fuzzy!—Class probabilities and floristic gradients as alternatives to crisp mapping for remote sensing of vegetation

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    Mapping vegetation as hard classes based on remote sensing data is a frequently applied approach, even though this crisp, categorical representation is not in line with nature\u27s fuzziness. Gradual transitions in plant species composition in ecotones and faint compositional differences across different patches are thus poorly described in the resulting maps. Several concepts promise to provide better vegetation maps. These include (1) fuzzy classification (a.k.a. soft classification) that takes the probability of an image pixel\u27s class membership into account and (2) gradient mapping based on ordination, which describes plant species composition as a floristic continuum and avoids a categorical description of vegetation patterns. A systematic and comprehensive comparison of these approaches is missing to date. This paper hence gives an overview of the state of the art in fuzzy classification and gradient mapping and compares the approaches in a case study. The advantages and disadvantages of the approaches are discussed and their performance is compared to hard classification (a.k.a. crisp or boolean classification). Gradient mapping best conserves the information in the original data and does not require an a priori categorization. Fuzzy classification comes close in terms of information loss and likewise preserves the continuous nature of vegetation, however, still relying on a priori classification. The need for a priori classification may be a disadvantage or, in other cases, an advantage because it allows using categorical input data instead of the detailed vegetation records required for ordination. Both approaches support spatially explicit accuracy analyses, which further improves the usefulness of the output maps. Gradient mapping and fuzzy classification offer various advantages over hard classification, can always be transformed into a crisp map and are generally applicable to various data structures. We thus recommend the use of these approaches over hard classification for applications in ecological research

    The Refractive Index of Amorphous and Crystalline Water Ice in the UV-vis

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    Laboratory astrophysics and astrochemistryLaboratory astrophysics and astrochemistr

    POLG2 deficiency causes adult-onset syndromic sensory neuropathy, ataxia and parkinsonism

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    Objective: Mitochondrial dysfunction plays a key role in the pathophysiology of neurodegenerative disorders such as ataxia and Parkinson's disease. We describe an extended Belgian pedigree where seven individuals presented with adult-onset cerebellar ataxia, axonal peripheral ataxic neuropathy, and tremor, in variable combination with parkinsonism, seizures, cognitive decline, and ophthalmoplegia. We sought to identify the underlying molecular etiology and characterize the mitochondrial pathophysiology of this neurological syndrome. Methods: Clinical, neurophysiological, and neuroradiological evaluations were conducted. Patient muscle and cultured fibroblasts underwent extensive analyses to assess mitochondrial function. Genetic studies including genome-wide sequencing were conducted. Results: Hallmarks of mitochondrial dysfunction were present in patients' tissues including ultrastructural anomalies of mitochondria, mosaic cytochrome c oxidase deficiency, and multiple mtDNA deletions. We identified a splice acceptor variant in POLG2, c.970-1G>C, segregating with disease in this family and associated with a concomitant decrease in levels of POLG2 protein in patient cells. Interpretation: This work extends the clinical spectrum of POLG2 deficiency to include an overwhelming, adult-onset neurological syndrome that includes cerebellar syndrome, peripheral neuropathy, tremor, and parkinsonism. We therefore suggest to include POLG2 sequencing in the evaluation of ataxia and sensory neuropathy in adults, especially when it is accompanied by tremor or parkinsonism with white matter disease. The demonstration that deletions of mtDNA resulting from autosomal-dominant POLG2 variant lead to a monogenic neurodegenerative multicomponent syndrome provides further evidence for a major role of mitochondrial dysfunction in the pathomechanism of nonsyndromic forms of the component neurodegenerative disorders

    Will Remote Sensing Shape the Next Generation of Species Distribution Models?

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    Two prominent limitations of species distribution models (SDMs) are spatial biases in existing occurrence data and a lack of spatially explicit predictor variables to fully capture habitat characteristics of species. Can existing and emerging remote sensing technologies meet these challenges and improve future SDMs? We believe so. Novel products derived from multispectral and hyperspectral sensors, as well as future Light Detection and Ranging (LiDAR) and RADAR missions, may play a key role in improving model performance. In this perspective piece, we demonstrate how modern sensors onboard satellites, planes and unmanned aerial vehicles are revolutionizing the way we can detect and monitor both plant and animal species in terrestrial and aquatic ecosystems as well as allowing the emergence of novel predictor variables appropriate for species distribution modeling. We hope this interdisciplinary perspective will motivate ecologists, remote sensing experts and modelers to work together for developing a more refined SDM framework in the near future
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