3,313 research outputs found
On the nature of bias and defects in the software specification process
Implementation bias in a specification is an arbitrary constraint in the solution space. This paper describes the problem of bias. Additionally, this paper presents a model of the specification and design processes describing individual subprocesses in terms of precision/detail diagrams and a model of bias in multi-attribute software specifications. While studying how bias is introduced into a specification we realized that software defects and bias are dual problems of a single phenomenon. This was used to explain the large proportion of faults found during the coding phase at the Software Engineering Laboratory at NASA/GSFC
Multisystem proteinopathy due to a homozygous p.Arg159His VCP mutation : a tale of the unexpected
ObjectiveTo assess the clinical, radiologic, myopathologic, and proteomic findings in a patient manifesting a multisystem proteinopathy due to a homozygous valosin-containing protein gene (VCP) mutation previously reported to be pathogenic in the heterozygous state.MethodsWe studied a 36-year-old male index patient and his father, both presenting with progressive limb-girdle weakness. Muscle involvement was assessed by MRI and muscle biopsies. We performed whole-exome sequencing and Sanger sequencing for segregation analysis of the identified p.Arg159His VCP mutation. To dissect biological disease signatures, we applied state-of-the-art quantitative proteomics on muscle tissue of the index case, his father, 3 additional patients with VCP-related myopathy, and 3 control individuals.ResultsThe index patient, homozygous for the known p.Arg159His mutation in VCP, manifested a typical VCP-related myopathy phenotype, although with a markedly high creatine kinase value and a relatively early disease onset, and Paget disease of bone. The father exhibited a myopathy phenotype and discrete parkinsonism, and multiple deceased family members on the maternal side of the pedigree displayed a dementia, parkinsonism, or myopathy phenotype. Bioinformatic analysis of quantitative proteomic data revealed the degenerative nature of the disease, with evidence suggesting selective failure of muscle regeneration and stress granule dyshomeostasis.ConclusionWe report a patient showing a multisystem proteinopathy due to a homozygous VCP mutation. The patient manifests a severe phenotype, yet fundamental disease characteristics are preserved. Proteomic findings provide further insights into VCP-related pathomechanisms
Gene therapy in Duchenne muscular dystrophy: Identifying and preparing for the challenges ahead
Direct evidence for significant spin-polarization of EuS in Co/EuS multilayers at room temperature
The new era of spintronics promises the development of nanodevices, where the
electron spin will be used to store information and charge currents will be
replaced by spin currents. For this, ferromagnetic semiconductors at room
temperature are needed. We report on significant room-temperature spin
polarization of EuS in Co/EuS multilayers recorded by x-ray magnetic circular
dichroism (XMCD). The films were found to contain a mixture of divalent and
trivalent europium, but only Eu11 is responsible for the ferromagnetic
behavior of EuS. The magnetic XMCD signal of Eu at room temperature could
unambiguously be assigned to magnetic ordering of EuS and was found to be only
one order of magnitude smaller than that at 2.5 K. The room temperature
magnetic moment of EuS is as large as the one of bulk ferromagnetic Ni. Our
findings pave the path for fabrication of room–temperature spintronic devices
using spin polarized EuS layers
Proximity effecs and curie temperature enhancement in Co/EuS and Fe/EuS multilayers
Two identical Co/EuS and Fe/EuS multilayers of six periods each and with
individual layers of about 4 nm thick are grown by e-beam evaporation under
ultrahigh vacuum conditions. The films show polycrystalline structure with a
grain size limited by the individual layer thickness. Both multilayers consist
of almost continuous layers with some roughness. The surface peak-to-peak
roughness is about 4–5 nm. Magnetization measurements and calculations of the
loops based on a Stoner–Wohlfarth-like model allow us to determine the direct
antiferromagnetic exchange coupling constant between the 3d metal and EuS at 5
K. Both samples show strong enhancement of the Curie temperature of EuS up to
at least 50 K with a EuS magnetization tail, which persists up to about 100 K.
The J = 7/2 character of the EuS layers is shown to be responsible for the
large Curie temperature enhancement
Lifetimes of image-potential states on copper surfaces
The lifetime of image states, which represent a key quantity to probe the
coupling of surface electronic states with the solid substrate, have been
recently determined for quantum numbers on Cu(100) by using
time-resolved two-photon photoemission in combination with the coherent
excitation of several states (U. H\"ofer et al, Science 277, 1480 (1997)). We
here report theoretical investigations of the lifetime of image states on
copper surfaces. We evaluate the lifetimes from the knowledge of the
self-energy of the excited quasiparticle, which we compute within the GW
approximation of many-body theory. Single-particle wave functions are obtained
by solving the Schr\"odinger equation with a realistic one-dimensional model
potential, and the screened interaction is evaluated in the random-phase
approximation (RPA). Our results are in good agreement with the experimentally
determined decay times.Comment: 4 pages, 1 figure, to appear in Phys. Rev. Let
Quantitative Imaging of Protein-Protein Interactions by Multiphoton Fluorescence Lifetime Imaging Microscopy using a Streak camera
Fluorescence Lifetime Imaging Microscopy (FLIM) using multiphoton excitation
techniques is now finding an important place in quantitative imaging of
protein-protein interactions and intracellular physiology. We review here the
recent developments in multiphoton FLIM methods and also present a description
of a novel multiphoton FLIM system using a streak camera that was developed in
our laboratory. We provide an example of a typical application of the system in
which we measure the fluorescence resonance energy transfer between a
donor/acceptor pair of fluorescent proteins within a cellular specimen.Comment: Overview of FLIM techniques, StreakFLIM instrument, FRET application
Spin-orbit splitting of image states
We quantify the effect of the spin-orbit interaction on the Rydberg-like
series of image state electrons at the (111) and (001) surface of Ir, Pt and
Au. Using relativistic multiple-scattering methods we find Rashba-like
dispersions with Delta E(K)=gamma K with values of gamma for n=1 states in the
range 38-88 meV Angstrom. Extending the phase-accumulation model to include
spin-orbit scattering we find that the splittings vary like 1/(n+a)^3 where a
is the quantum defect and that they are related to the probability of spin-flip
scattering at the surface. The splittings should be observable experimentally
being larger in magnitude than some exchange-splittings that have been resolved
by inverse photoemission, and are comparable to linewidths from inelastic
lifetimes.Comment: 10 pages, 4 figure
Nonlinear Magneto-Optics of Fe Monolayers from first principles: Structural dependence and spin-orbit coupling strength
We calculate the nonlinear magneto-optical response of free-standing fcc
(001), (110) and (111) oriented Fe monolayers. The bandstructures are
determined from first principles using a full-potential LAPW method with the
additional implementation of spin-orbit coupling. The variation of the
spin-orbit coupling strength and the nonlinear magneto-optical spectra upon
layer orientation are investigated. We find characteristic differences which
indicate an enhanced sensitivity of nonlinear magneto-optics to surface
orientation and variation of the in-plane lattice constants. In particular the
crossover from onedimensional stripe structures to twodimensional films of
(111) layers exhibits a clean signature in the nonlinear Kerr-spectra and
demonstrates the versatility of nonlinear magneto-optics as a tool for in situ
thin-film analysis.Comment: 28 pages, RevTeX, psfig, submitted to PR
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