434 research outputs found

    Understanding consumer demand for new transport technologies and services, and implications for the future of mobility

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    The transport sector is witnessing unprecedented levels of disruption. Privately owned cars that operate on internal combustion engines have been the dominant modes of passenger transport for much of the last century. However, recent advances in transport technologies and services, such as the development of autonomous vehicles, the emergence of shared mobility services, and the commercialization of alternative fuel vehicle technologies, promise to revolutionise how humans travel. The implications are profound: some have predicted the end of private car dependent Western societies, others have portended greater suburbanization than has ever been observed before. If transport systems are to fulfil current and future needs of different subpopulations, and satisfy short and long-term societal objectives, it is imperative that we comprehend the many factors that shape individual behaviour. This chapter introduces the technologies and services most likely to disrupt prevailing practices in the transport sector. We review past studies that have examined current and future demand for these new technologies and services, and their likely short and long-term impacts on extant mobility patterns. We conclude with a summary of what these new technologies and services might mean for the future of mobility.Comment: 15 pages, 0 figures, book chapte

    Hydrologically-driven crustal stresses and seismicity in the New Madrid Seismic Zone

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    The degree to which short-term non-tectonic processes, either natural and anthropogenic, influence the occurrence of earthquakes in active tectonic settings or ‘stable’ plate interiors, remains a subject of debate. Recent work in plate-boundary regions demonstrates the capacity for long-wavelength changes in continental water storage to produce observable surface deformation, induce crustal stresses and modulate seismicity rates. Here we show that a significant variation in the rate of microearthquakes in the intraplate New Madrid Seismic Zone at annual and multi-annual timescales coincides with hydrological loading in the upper Mississippi embayment. We demonstrate that this loading, which results in geodetically observed surface deformation, induces stresses within the lithosphere that, although of small amplitude, modulate the ongoing seismicity of the New Madrid region. Correspondence between surface deformation, hydrological loading and seismicity rates at both annual and multi-annual timescales indicates that seismicity variations are the direct result of elastic stresses induced by the water load

    Cell-based analysis of CAD variants identifies individuals likely to benefit from uridine therapy.

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    PURPOSE: Pathogenic autosomal recessive variants in CAD, encoding the multienzymatic protein initiating pyrimidine de novo biosynthesis, cause a severe inborn metabolic disorder treatable with a dietary supplement of uridine. This condition is difficult to diagnose given the large size of CAD with over 1000 missense variants and the nonspecific clinical presentation. We aimed to develop a reliable and discerning assay to assess the pathogenicity of CAD variants and to select affected individuals that might benefit from uridine therapy. METHODS: Using CRISPR/Cas9, we generated a human CAD-knockout cell line that requires uridine supplements for survival. Transient transfection of the knockout cells with recombinant CAD restores growth in absence of uridine. This system determines missense variants that inactivate CAD and do not rescue the growth phenotype. RESULTS: We identified 25 individuals with biallelic variants in CAD and a phenotype consistent with a CAD deficit. We used the CAD-knockout complementation assay to test a total of 34 variants, identifying 16 as deleterious for CAD activity. Combination of these pathogenic variants confirmed 11 subjects with a CAD deficit, for whom we describe the clinical phenotype. CONCLUSIONS: We designed a cell-based assay to test the pathogenicity of CAD variants, identifying 11 CAD-deficient individuals who could benefit from uridine therapy

    The interior of the SNR RX J0852.0-4622 (Vela Jr) at radio wavelengths

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    Aim: We observed the center of the supernova remnant Vela Jr in radio continuum in order to search for a counterpart to the compact central X-ray source CXOU J085201.4-461753, possibly a neutron star candidate which could be the remnant of the supernova explosion. Method: Observations were made with the Australia Telescope Compact Array at 13 and 20 cm. Spectral indices were obtained using flux density correlations of the data which were spatially filtered to have the same u-v coverage. A multiwavelength search for counterparts to the compact central X-ray source was made. Results: We compiled a new catalogue of 31 small diameter radio sources, including the previously known source PMN J0853-4620, listing the integrated flux densities at 20 cm and, for half of the sources, the flux densities at 13 cm with the corresponding spectral indices. All sources are unresolved at the present angular resolution except for Source 18, which is clearly elongated and lies strikingly close to CXOU J085201.4-461753. Our observations show no evidence for the existence of a pulsar wind driven nebula associated with the point X-ray source. Furthermore, Source 18 has a thermal spectrum with index +0.8 +/- 0.4, and appears to be the counterpart of the optical source Wray 16-30. In spite of the absence of OIII emission lines as reported in the literature, we find that this object could be explained as a low emission planetary nebula belonging to the ``butterfly'' morphological class. Conclusions: We conclude that if the radio source 18 is actually a planetary nebula, then CXOU J085201.4-461753 is more likely to be related to it rather than to Vela Jr.Comment: 9 pages, 4 figures, to appear in Astronomy and Astrophysic

    Expanding Clinical Presentations Due to Variations in THOC2 mRNA Nuclear Export Factor

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    Multiple TREX mRNA export complex subunits (e.g., THOC1, THOC2, THOC5, THOC6, THOC7) have now been implicated in neurodevelopmental disorders (NDDs), neurodegeneration and cancer. We previously implicated missense and splicing-defective THOC2 variants in NDDs and a broad range of other clinical features. Here we report 10 individuals from nine families with rare missense THOC2 variants including the first case of a recurrent variant (p.Arg77Cys), and an additional individual with an intragenic THOC2 microdeletion (Del-Ex37-38). Ex vivo missense variant testing and patient-derived cell line data from current and published studies show 9 of the 14 missense THOC2 variants result in
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