2,024 research outputs found

    Biomechanical properties of bone in a mouse model of Rett syndrome

    Get PDF
    Rett syndrome (RTT) is an X-linked genetic disorder and a major cause of intellectual disability in girls. Mutations in the methyl-CpG binding protein 2 (<i>MECP2</i>) gene are the primary cause of the disorder. Despite the dominant neurological phenotypes, <i>MECP2</i> is expressed ubiquitously throughout the body and a number of peripheral phenotypes such as scoliosis, reduced bone mineral density and skeletal fractures are also common and important clinical features of the disorder. In order to explore whether MeCP2 protein deficiency results in altered structural and functional properties of bone and to test the potential reversibility of any defects, we have conducted a series of histological, imaging and biomechanical tests of bone in a functional knockout mouse model of RTT. Both hemizygous <i>Mecp2</i><sup>stop/y</sup> male mice in which <i>Mecp2</i> is silenced in all cells and female <i>Mecp2</i><sup>stop/+</sup> mice in which <i>Mecp2</i> is silenced in ~ 50% of cells as a consequence of random X-chromosome inactivation, revealed significant reductions in cortical bone stiffness, microhardness and tensile modulus. Microstructural analysis also revealed alterations in both cortical and cancellous femoral bone between wild-type and MeCP2-deficient mice. Furthermore, unsilencing of <i>Mecp2</i> in adult mice cre-mediated stop cassette deletion resulted in a restoration of biomechanical properties (stiffness, microhardness) towards wild-type levels. These results show that MeCP2-deficiency results in overt, but potentially reversible, alterations in the biomechanical integrity of bone and highlights the importance of targeting skeletal phenotypes in considering the development of pharmacological and gene-based therapies

    New Measurement of Compton Scattering from the Deuteron and an Improved Extraction of the Neutron Electromagnetic Polarizabilities

    Get PDF
    The electromagnetic polarizabilities of the nucleon are fundamental properties that describe its response to external electric and magnetic fields. They can be extracted from Compton-scattering data --- and have been, with good accuracy, in the case of the proton. In contradistinction, information for the neutron requires the use of Compton scattering from nuclear targets. Here we report a new measurement of elastic photon scattering from deuterium using quasimonoenergetic tagged photons at the MAX IV Laboratory in Lund, Sweden. These first new data in more than a decade effectively double the world dataset. Their energy range overlaps with previous experiments and extends it by 20 MeV to higher energies. An analysis using Chiral Effective Field Theory with dynamical \Delta(1232) degrees of freedom shows the data are consistent with and within the world dataset. After demonstrating that the fit is consistent with the Baldin sum rule, extracting values for the isoscalar nucleon polarizabilities and combining them with a recent result for the proton, we obtain the neutron polarizabilities as \alpha_n = [11.55 +/- 1.25(stat) +/- 0.2(BSR) +/- 0.8(th)] X 10^{-4} fm^3 and \beta_n = [3.65 -/+ 1.25(stat) +/- 0.2(BSR) -/+ 0.8(th)] X 10^{-4} fm3, with \chi^2 = 45.2 for 44 degrees of freedom.Comment: 6 pages, 3 figures, comments from Physical Review Letters Referees addresse

    Sustainable wastewater management in Indonesia's fish processing industry: Bringing governance into scenario analysis

    Get PDF
    The government of Indonesia has pledged to meet ambitious greenhouse gas mitigation goals in its Nationally Determined Contribution as well as reduce water pollution through its water management policies. A set of technologies could conceivably help achieving these goals simultaneously. However, the installation and widespread application of these technologies will require knowledge on how governance affects the implementation of existing policies as well as cooperation across sectors, administrative levels, and stakeholders. This paper integrates key governance variables--involving enforcement capacity, institutional coordination and multi-actor networks--into an analysis of the potential impacts on greenhouse gases and chemical oxygen demand in seven wastewater treatment scenarios for the fish processing industry in Indonesia. The analysis demonstrates that there is an increase of 24% in both CH4 and CO2 emissions between 2015 and 2030 in the business-as-usual scenario due to growth in production volumes. Interestingly, in scenarios focusing only on strengthening capacities to enforce national water policies, expected total greenhouse gas emissions are about five times higher than in the business-as-usual in 2030; this is due to growth in CH4 emissions during the handling and landfilling of sludge, as well as in CO2 generated from the electricity required for wastewater treatment. In the scenarios where there is significant cooperation across sectors, administrative levels, and stakeholders to integrate climate and water goals, both estimated chemical oxygen demand and CH4 emissions are considerably lower than in the business-as-usual and the national water policy scenarios
    • …
    corecore