Rare manifestation of a c.290 C\u3eT, p.Gly97Glu VCP mutation

Introduction. The valosin-containing protein (VCP) regulates several distinct cellular processes. Consistent with this, VCP mutations manifest variable clinical phenotypes among and within families and are a diagnostic challenge. Methods. A 60-year-old man who played ice hockey into his 50’s was evaluated by electrodiagnostics, muscle biopsy, and molecular genetics. Results. With long-standing pes cavus and toe walking, our patient developed progressive weakness, cramps, memory loss, and paresthesias at age 52. An axonal sensorimotor neuropathy was found upon repeated testing at age 58. Neuropathic histopathology was present in the quadriceps, and exome sequencing revealed the VCP mutation c.290 C>T, p.Gly97Glu. Conclusions. Our patient reflects the clinical heterogeneity of VCP mutations, as his neurological localization is a spectrum between a lower motor neuron disorder and a hereditary axonal peripheral neuropathy such as CMT2. Our case demonstrates a rare manifestation of the c.290 C>T, pGly97Glu VCP mutation

Hardness of porous nanocrystalline Co-Ni electrodeposits

The Hall-Petch relationship can fail when the grain size is below a critical value of tens of nanometres. This occurs particularly for coatings having porous surfaces. In this study, electrodeposited nanostructured Co-Ni coatings from four different nickel electroplating baths having grain sizes in the range of 11-23 nm have been investigated. The finest grain size, approximately 11 nm, was obtained from a coating developed from the nickel sulphate bath. The Co-Ni coatings have a mixed face centred cubic and hexagonal close-packed structures with varying surface morphologies and different porosities. A cluster-pore mixture model has been proposed by considering no contribution from pores to the hardness. As the porosity effect was taken into consideration, the calculated pore-free hardness is in agreement with the ordinary Hall-Petch relationship even when the grain size is reduced to 11 nm for the Co-Ni coatings with 77±2 at% cobalt. The present model was applied to other porous nanocrystalline coatings, and the Hall-Petch relationship was maintained. © 2013 The Korean Institute of Metals and Materials and Springer Science+Business Media Dordrecht. © KIM and Springer

Choice of activity-intensity classification thresholds impacts upon accelerometer-assessed physical activity-health relationships in children

It is unknown whether using different published thresholds (PTs) for classifying physical activity (PA) impacts upon activity-health relationships. This study explored whether relationships between PA (sedentary [SED], light PA [LPA], moderate PA [MPA], moderate-to-vigorous PA, vigorous PA [VPA]) and health markers differed in children when classified using three different PTs

Does femtosecond time-resolved second-harmonic generation probe electron temperatures at surfaces?

Femtosecond pump-probe second-harmonic generation (SHG) and transient linear reflectivity measurements were carried out on polycrystalline Cu, Ag and Au in air to analyze whether the electron temperature affects Fresnel factors or nonlinear susceptibilities, or both. Sensitivity to electron temperatures was attained by using photon energies near the interband transition threshold. We find that the nonlinear susceptibility carries the electron temperature dependence in case of Ag and Au, while for Cu the dependence is in the Fresnel factors. This contrasting behavior emphasizes that SHG is not a priori sensitive to electron dynamics at surfaces or interfaces, notwithstanding its cause.Comment: 11 pages, 4 figure

On Synchronization in a Lattice Model of Pulse-Coupled Oscillators

We analyze the collective behavior of a lattice model of pulse-coupled oscillators. By means of computer simulations we find the relation between the intrinsic dynamics of each member of the population and their mutual interaction that ensures, in a general context, the existence of a fully synchronized regime. This condition turns out to be the same than the obtained for the globally coupled population. When the condition is not completely satisfied we find different spatial structures. This also gives some hints about self-organized criticality.Comment: 4 pages, RevTex, 1 PostScript available upon request, To appear in Phys. Rev. Let

A High Statistics Search for Electron-Neutrino --> Tau-Neutrino Oscillations

We present new limits on nu_e to nu_tau and nu_e to nu_sterile oscillations by searching for electron neutrino dissappearance in the high-energy wide-band CCFR neutrino beam. Sensitivity to nu_tau appearance comes from tau decay modes in which a large fraction of the energy deposited is electromagnetic. The beam is composed primarily of muon neutrinos but this analysis uses the 2.3% electron neutrino component of the beam. Electron neutrino energies range from 30 to 600 GeV and flight lengths vary from 0.9 to 1.4 km. This limit improves the sensitivity of existing limits and obtains a lowest 90% confidence upper limit in sin**2(2*alpha) of 9.9 x 10**(-2) at delta-m**2 of 125 eV**2.Comment: submitted to Phys. Rev. D. Rapid Com

New Measurements of Nucleon Structure Functions from the CCFR/NuTeV Collaboration

We report on the extraction of the structure functions F_2 and Delta xF_3 = xF_3nu-xF_3nubar from CCFR neutrino-Fe and antineutrino-Fe differential cross sections. The extraction is performed in a physics model independent (PMI) way. This first measurement for Delta xF_3, which is useful in testing models of heavy charm production, is higher than current theoretical predictions. The F_2 (PMI) values measured in neutrino and muon scattering are in good agreement with the predictions of Next to Leading Order PDFs (using massive charm production schemes), thus resolving the long-standing discrepancy between the two sets of data.Comment: 5 pages. Presented by Arie Bodek at the CIPNAP2000 Conference, Quebec City, May 200