Upper Limit on the Cosmological Gamma-ray Background

We show that the current extragalactic gamma-ray background (EGB) measurement below 100 GeV sets an upper limit on EGB itself at very high energy (VHE) above 100 GeV. The limit is conservative for the electromagnetic cascade emission from VHE EGB interacting with the cosmic microwave-to-optical background radiation not to exceed the current EGB measurement. The cascade component fits the measured VHE EGB spectrum rather well. However, once we add the contribution from known source classes, the Fermi VHE EGB observation exceeds or even violates the limit, which is approximated as E^2dN/dE < 4.5x10^-5 (E/100 GeV)^-0.7 MeV/cm^2/s/sr. The upper limit above 100 GeV is useful in the future to probe the EGB origin and the new physics like axion-like particles and Lorentz-invariance violation.Comment: 8 pages, 8 figures, accepted for publication in PRD, Interested readers may wish to consult the recent paper by Murase, Beacom and Takami (arXiv:1205.5755

Observation of thundercloud-related gamma rays and neutrons in Tibet

During the 2010 rainy season in Yangbajing (4300 m above sea level) in Tibet, China, a long-duration count enhancement associated with thunderclouds was detected by a solar-neutron telescope and neutron monitors installed at the Yangbajing Comic Ray Observatory. The event, lasting for ∼40  min, was observed on July 22, 2010. The solar-neutron telescope detected significant γ-ray signals with energies >40  MeV in the event. Such a prolonged high-energy event has never been observed in association with thunderclouds, clearly suggesting that electron acceleration lasts for 40 min in thunderclouds. In addition, Monte Carlo simulations showed that >10  MeV γ rays largely contribute to the neutron monitor signals, while >1  keV neutrons produced via a photonuclear reaction contribute relatively less to the signals. This result suggests that enhancements of neutron monitors during thunderstorms are not necessarily clear evidence for neutron production, as previously thought

Electron transport across a quantum wire in the presence of electron leakage to a substrate

We investigate electron transport through a mono-atomic wire which is tunnel coupled to two electrodes and also to the underlying substrate. The setup is modeled by a tight-binding Hamiltonian and can be realized with a scanning tunnel microscope (STM). The transmission of the wire is obtained from the corresponding Green's function. If the wire is scanned by the contacting STM tip, the conductance as a function of the tip position exhibits oscillations which may change significantly upon increasing the number of wire atoms. Our numerical studies reveal that the conductance depends strongly on whether or not the substrate electrons are localized. As a further ubiquitous feature, we observe the formation of charge oscillations.Comment: 7 pages, 7 figure

Spectroscopic ellipsometry and polarimetry for materials and systems analysis at the nanometer scale: state-of-the-art, potential, and perspectives

This paper discusses the fundamentals, applications, potential, limitations, and future perspectives of polarized light reflection techniques for the characterization of materials and related systems and devices at the nanoscale. These techniques include spectroscopic ellipsometry, polarimetry, and reflectance anisotropy. We give an overview of the various ellipsometry strategies for the measurement and analysis of nanometric films, metal nanoparticles and nanowires, semiconductor nanocrystals, and submicron periodic structures. We show that ellipsometry is capable of more than the determination of thickness and optical properties, and it can be exploited to gain information about process control, geometry factors, anisotropy, defects, and quantum confinement effects of nanostructures

Physical training in boys with Duchenne Muscular Dystrophy: the protocol of the No Use is Disuse study

Contains fulltext : 89740.pdf (publisher's version ) (Open Access)BACKGROUND: "Use it or lose it" is a well known saying which is applicable to boys with Duchenne Muscular Dystrophy (DMD). Besides the direct effects of the muscular dystrophy, the increasing effort to perform activities, the fear of falling and the use of personal aids indirectly impair leg and arm functions as a result of disuse. Physical training could oppose this secondary physical deterioration. The No Use is Disuse (NUD) study is the first study in human subjects with DMD that will examine whether a low-intensity physical training is beneficial in terms of preservation of muscle endurance and functional abilities. The study consists of two training intervention studies: study 1 "Dynamic leg and arm training for ambulant and recently wheelchair-dependent boys with DMD and, study 2 "Functional training with arm support for boys with DMD who have been confined to a wheelchair for several years". This paper describes the hypotheses and methods of the NUD study. METHODS: Study 1 is an explorative randomized controlled trial with multiple baseline measurements. Thirty boys with a DNA-established diagnosis of DMD will be included. The intervention consists of a six-months physical training during which boys train their legs and arms with active and/or assisted cycling training equipment. The primary study outcomes are muscle endurance and functional abilities, assessed with a Six-Minute Bicycle Test and the Motor Function Measure. Study 2 has a within-group repeated measurements design and will include ten boys with DMD who have already been confined to a wheelchair for several years. The six-months physical training program consists of 1) a computer-assisted training and 2) a functional training with an arm support. The primary study outcome is functional abilities of the upper extremity, assessed with the Action Research Arm Test. DISCUSSION: The NUD study will fill part of the gap in the current knowledge about the possible effects of training in boys with DMD and will increase insight into what type of exercise should be recommended to boys with DMD. The study will finish at the end of 2010 and results are expected in 2011. TRIAL REGISTRATION: The Netherlands National Trial Register1631

COL4A1 Mutations Cause Ocular Dysgenesis, Neuronal Localization Defects, and Myopathy in Mice and Walker-Warburg Syndrome in Humans

Muscle-eye-brain disease (MEB) and Walker Warburg Syndrome (WWS) belong to a spectrum of autosomal recessive diseases characterized by ocular dysgenesis, neuronal migration defects, and congenital muscular dystrophy. Until now, the pathophysiology of MEB/WWS has been attributed to alteration in dystroglycan post-translational modification. Here, we provide evidence that mutations in a gene coding for a major basement membrane protein, collagen IV alpha 1 (COL4A1), are a novel cause of MEB/WWS. Using a combination of histological, molecular, and biochemical approaches, we show that heterozygous Col4a1 mutant mice have ocular dysgenesis, neuronal localization defects, and myopathy characteristic of MEB/WWS. Importantly, we identified putative heterozygous mutations in COL4A1 in two MEB/WWS patients. Both mutations occur within conserved amino acids of the triple-helix-forming domain of the protein, and at least one mutation interferes with secretion of the mutant proteins, resulting instead in intracellular accumulation. Expression and posttranslational modification of dystroglycan is unaltered in Col4a1 mutant mice indicating that COL4A1 mutations represent a distinct pathogenic mechanism underlying MEB/WWS. These findings implicate a novel gene and a novel mechanism in the etiology of MEB/WWS and expand the clinical spectrum of COL4A1-associated disorders

7th Drug hypersensitivity meeting: part two

No abstract availabl