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81.
82.
TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis   总被引:1,自引:0,他引:1  
Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disorder characterized pathologically by ubiquitinated TAR DNA binding protein (TDP-43) inclusions. The function of TDP-43 in the nervous system is uncertain, and a mechanistic role in neurodegeneration remains speculative. We identified neighboring mutations in a highly conserved region of TARDBP in sporadic and familial ALS cases. TARDBPM337V segregated with disease within one kindred and a genome-wide scan confirmed that linkage was restricted to chromosome 1p36, which contains the TARDBP locus. Mutant forms of TDP-43 fragmented in vitro more readily than wild type and, in vivo, caused neural apoptosis and developmental delay in the chick embryo. Our evidence suggests a pathophysiological link between TDP-43 and ALS.  相似文献   
83.
  1. Taxonomic and functional trajectories of benthic assemblages were studied in shallow soft-bottom sediments in the bay of Saint-Brieuc (Western English Channel). Changes were assessed at different spatial and temporal scales using a macrobenthic dataset based on 38 stations sampled in 1987 and 2019, coupled with data from one station sampled annually between 2005 and 2019 as part of the European Water Framework Directive monitoring programme.
  2. Taxonomic trajectories indicated 1) changes in the structure and distribution of benthic assemblages, 2) an homogenization of the assemblages, and 3) significant functional shifts.
  3. Fishing activities and nutrient enrichment are probably strong drivers of the observed changes, as suggested by the higher mortality rate of fragile, flexible, tubiculous and burrowing species, and the increase in the abundance of opportunistic species in the assemblages. Certain populations of macroinvertebrates seem jointly controlled by climate change and by the aforementioned local factors of disturbance.
  4. The Community Trajectory Analysis framework appears as a new and interesting method to track ecological changes in marine ecosystems by measuring change with respect to a baseline state, to help define ecological recovery (station returning to the initial ecological state) and departure (station presenting increased changes over time), and to analyse trajectory similarity.
  5. According to the degradation of habitat over time, we identify the need for the implementation of knowledge-based conservation strategies, especially within Natura 2000 sites.
  相似文献   
84.
Small‐scale fisheries are recognised as making important contributions to nutrition and economic development despite a lack of accurate quantitative information on catches and consumption. While direct measurement remains the most appropriate way of collecting such data, it is impractical at large scales. Instead, household surveys based upon informant recall of fish caught and/or consumed are frequently used. However, the accuracy of weight recall by informants (even over short recall periods) has not been established. Using data from household surveys, the accuracy and precision of catch and consumption estimates derived from: (a) asking informants to recall weights of fish caught and (b) asking respondents to recall lengths of fish caught and converting to weight were tested. Length‐based methods, using visual aids to assist recall, were more accurate, precise and correctable. These methods could be useful for catch estimation, especially where fish are processed, sold or consumed shortly after capture.  相似文献   
85.
Between 1950 and 1989, marine fisheries catch in the open‐ocean and deep‐sea beyond 200 nautical miles from shore increased by a factor of more than 10. While high seas catches have since plateaued, fishing effort continues to increase linearly. The combination of increasing effort and illegal, unreported and unregulated (IUU) fishing has led to overfishing of target stocks and declines in biodiversity. To improve management, there have been numerous calls to increase monitoring, control and surveillance (MCS). However, MCS has been unevenly implemented, undermining efforts to sustainably use high seas and straddling stocks and protect associated species and ecosystems. The United Nations General Assembly is currently negotiating a new international treaty for the conservation and sustainable use of biodiversity beyond national jurisdiction (BBNJ). The new treaty offers an excellent opportunity to address discrepancies in how MCS is applied across regional fisheries management organizations (RFMOs). This paper identifies ways that automatic identification system (AIS) data can inform MCS on the high seas and thereby enhance conservation and management of biodiversity beyond national jurisdictions. AIS data can be used to (i) identify gaps in governance to underpin the importance of a holistic scope for the new agreement; (ii) monitor area‐based management tools; and (iii) increase the capacity of countries and RFMOs to manage via the technology transfer. Any new BBNJ treaty should emphasize MCS and the role of electronic monitoring including the use of AIS data, as well as government–industry–civil society partnerships to ensure critically important technology transfer and capacity building.  相似文献   
86.
Given the choice of waiting for an adverse outcome or getting it over with quickly, many people choose the latter. Theoretical models of decision-making have assumed that this occurs because there is a cost to waiting-i.e., dread. Using functional magnetic resonance imaging, we measured the neural responses to waiting for a cutaneous electric shock. Some individuals dreaded the outcome so much that, when given a choice, they preferred to receive more voltage rather than wait. Even when no decision was required, these extreme dreaders were distinguishable from those who dreaded mildly by the rate of increase of neural activity in the posterior elements of the cortical pain matrix. This suggests that dread derives, in part, from the attention devoted to the expected physical response and not simply from fear or anxiety. Although these differences were observed during a passive waiting procedure, they correlated with individual behavior in a subsequent choice paradigm, providing evidence for a neurobiological link between the experienced disutility of dread and subsequent decisions about unpleasant outcomes.  相似文献   
87.
Self-assembling materials are the building blocks of bottom-up nanofabrication processes, but they need to be templated to impose long-range order and eliminate defects. In this work, the self-assembly of a thin film of a spherical-morphology block copolymer is templated using an array of nanoscale topographical elements that act as surrogates for the minority domains of the block copolymer. The orientation and periodicity of the resulting array of spherical microdomains are governed by the commensurability between the block copolymer period and the template period and is accurately described by a free-energy model. This method, which forms high-spatial-frequency arrays using a lower-spatial-frequency template, will be useful in nanolithography applications such as the formation of high-density microelectronic structures.  相似文献   
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Pigeon circovirus (PiCV) infection and young pigeon disease syndrome (YPDS), associated with high morbidity and mortality, have been recognized in young racing pigeons from large portions of Central Europe. There exist a number of data indicating that YPDS is a consequence of PiCV infection and subsequent immunosuppression. In order to prove PiCV to be one of the crucial factors of YPDS, an experimental infection with PiCV was performed under controlled conditions. Twenty-four domestic pigeons (Columba livia forma domestica) were divided into two groups with 12 pigeons each; an infection group and a control group. All birds were between their fourth to eighth week of life. Pigeons in the infection group were infected both intramuscularly and orally with PiCV purified from naturally infected birds, while pigeons in the control group received a placebo. To test a possible influence of the PiCV infection on the immune system, the animals in both groups were vaccinated simultaneously, on the same day, against PMV-1 (Lasovac plus, IDT, Dessau-Tornau, Germany). Weekly virologic testing showed a viraemic period, and excretion of the infection virus, in pigeons in the infection group. Replication of PiCV could be proved on the basis of histologic findings of multiglobular inclusion bodies, mainly observed in macrophages of the bursa of Fabricius. A PiCV, genetically distinct from the experimental virus, was detected in the control group by polymerase chain reaction (PCR) testing, but any histologic findings comparable to the infection group were absent. None of the pigeons revealed clinical signs of illness, or hints that immunosuppression had occurred, regardless of their group. The absence of stressful conditions, considered as a trigger for the development of YPDS, may be responsible for the failure of disease reproduction in our infection model.  相似文献   
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