Functional analysis of Gscl in the pathogenesis of the DiGeorge and velocardiofacial syndromes

Gscl encodes a Goosecoid-related homeodomain protein that is expressed during mouse embryogenesis. In situ hybridization and immunohistochemistry studies show that Gscl is expressed in the pons region of the developing central nervous system and primordial germ cells. Gscl expression is also detected in a subset of adult tissues, including brain, eye, thymus, thyroid region, stomach, bladder and testis. Gscl is located within a region of the mouse genome that is syntenic with the region commonly deleted in DiGeorge and velocardiofacial syndrome (DGS/VCFS) patients. DGS/VCFS patients have craniofacial abnormalities, cardiac outflow defects and hypoplasia of the parathyroid gland and thymus due to haploinsufficiency of a gene or genes located within the deleted region. Thus, the genomic location of Gscl and its expression in a subset of the tissues affected in DGS/VCFS patients suggest that Gscl may contribute to the pathogenesis of DGS/VCFS. To determine the role of Gscl during mouse embryogenesis and in DGS/VCFS, we have deleted Gscl by gene targeting in mouse embryonic stem cells. Both Gscl heterozygous and Gscl null mice were normal and fertile, suggesting that Gscl is not a major factor in DGS/VCFS. Interestingly, expression of the adjacent Es2 gene in the pons region of Gscl null fetuses was absent, suggesting that mutations within the DGS/VCFS region can influence expression of adjacent genes. In addition, embryos that lacked both Gscl and the related Gsc gene appeared normal. These studies represent the first functional analysis of a DGS/VCFS candidate gene in vivo. These Gscl null mice will be an important genetic resource for crosses with other mouse models of the DGS/VCFS.     

Investigation of space-energy effects in the reactivity measurement by neutron noise with excore detectors in a reflected LWR

Practical application of the zero-crossing correlation method for measuring slightly-subcritical reactivities in a swimming-pool reactor required the use of detector locations in the reflector zone near to the core boundary. Experimental investigations of neutron-noise cross-power spectra showed significant deviations from the point-reactor model at higher frequencies (> 100 Hz). Nevertheless, the use of the point-reactor model was found to be a useful approach in the analysis of the zero-crossing correlation method, yielding results which agreed well with those obtained from the rod-drop method. The theoretical part of the work is concerned with a space-dependent model calculation in two-group diffusion theory to support the experimental findings. The model calculation can explain the trends observed in the neutron-noise spectra as well as the applicability of the point-reactor model to the zero-crossing correlation method. To obtain better insight, the calculations have been extended to neutron-noise spectra when one or both detectors are located in the core zone. In the case of a large core and widely-spaced detectors, with at least one detector in the core zone, a sink frequency appears in the spectra. This effect is well known in coupled-core kinetics.     

Comparison of Voxel-Based and Mesh-Based CFD Models for Aerosol Deposition on Complex Fibrous Filters

Aerosols represent a health risk since small droplets may enter the respiratory system and cause lung cancer, allergies, or diseases like COVID-19. In this work, an Eulerian-Lagrangian computational fluid dynamics model is used based on a voxel-based (GeoDict) and a mesh-based (StarCCM+) code. For evaluating accuracy and computational time of both models, fractional filtration efficiency and pressure drop are compared to an empirical solution for a single fiber and to experimental results for a complex 3D fibrous filter material. Simulation results of both methods are in good agreement with empirical and measurement results although the complex geometry of the fibers is captured more accurately by the unstructured mesh using the same resolution. Computing times are much faster using the voxel-based code.     

Requirement for Lim1 in head-organizer function

Lim1 is a homeobox gene expressed in the organizer region of mouse embryos. To investigate the role of Lim1 during embryogenesis, a targeted deletion of the Lim1 gene was generated in embryonic stem cells. Embryos homozygous for the null allele lacked anterior head structures but the remaining body axis developed normally. A partial secondary axis developed anteriorly in some mutant embryos. Lim1 is thus an essential regulator of the vertebrate head organizer.     

Thermal equilibration of fluids near the liquid-vapor critical point:3He and3He-4He mixtures

We study the temperature-equilibration process of fluids at constant volume in a thermal conductivity cell, where an initial temperature gradient relaxes to zero. The calculation is performed in the linear approximation for a pure fluid and a binary mixture. Near the critical point of the pure fluid, the adiabatic heating process, which takes place at constant volumeV, causes equilibration to proceed four times faster whenC _P /C _V 1 than for the process at constant pressureP. For the mixtures, the relaxation rate enhancement at constantV compared with constantP is restricted to a temperature region where the coupling between temperature and mass diffusion is small. The predictions are compared with experimental results for³He and for two³He-⁴He mixtures along their critical isochores. Finally, we discuss the thermal relaxation in the two-phase (liquid-gas) and one-phase (gas) regimes at the critical density, as measured with a conductivity and a calorimetry cell. The contrasting behavior for³He and a³He-⁴He mixture in these two regimes and under these different constraints is pointed out and discussed.     

Thermal response of a3He-superfluid-4He mixture

The response of a layer of superfluid mixture to an ac heat source,Q(t)=Q ₀ exp(it), is determined. In the low-frequency regime, the temperature response at the heated side of a superfluid layer is essentially identical to that of an ordinary fluid having a thermal conductivity _eff and a thermal diffusion coefficient ₀ /2. Here _eff is the effective conductivity of Khalatnikov, and ₀ is the diffusion coefficient of Griffin. At much higher frequencies, the results are more complicated. The low-frequency regime is defined in terms of the second sound velocityu ₂ by u ₂ ² / ₀ . The ac response function is valuable in a number of ways. It can be used to obtain the system response to more complicated time-dependent variations inQ such as step changes inQ. A knowledge of the response function in the low-frequency regime provides a mechanism for directly determining the Kapitza resistance in mixtures. Finally, a knowledge of the response function provides an additional opportunity to test two-fluid hydrodynamics. Alternative tests of superfluid hydrodynamics are of particular interest in light of recent experiments that show anomalous values for _eff in the low ³ He concentration limit     

Hydrodynamic heat flow in very dilute superfluid3He-4He mixtures

The superfluid hydrodynamics of heat flow is examined for very small mass concentrationsc of³He in⁴He in an effort to better understand recent results for the effective heat conductivity _eff, which appear to be in conflict with predictions. The full hydrodynamics contains a thermal boundary layer; within this layer the temperature and concentration gradients differ from those in the bulk fluid. An examination of finite heating effects based on the ansatz _eff c ^–p for smallc shows distinctly different behavior for experimental determinations of _eff whenp<1,p=1, andp>1. Thus, finite heating can be used as a probe to evaluate the exponentp.