2,351 research outputs found

    Thermal expansion and atomic displacement parameters of cubic KMgF3 perovskite determined by high-resolution neutron powder diffraction

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    The structure of KMgF3 has been determined by high-resolution neutron powder diffraction at 4.2 K, room temperature and at 10 K intervals from 373 K to 1223 K. The material remains cubic at all temperatures. The average volumetric coefficient of thermal expansion in the range 373-1223 K was found to be 7.11 (3) × 10-5 K-1. For temperatures between 4.2 and 1223 K, a second-order Grüneisen approximation to the zero-pressure equation of state, with the internal energy calculated via a Debye model, was found to fit well, with the following parameters: θD = 536 (9) K, Vo = 62.876 (6) Å3, Ko' = 6.5 (1) and (VoKo/Y') = 3.40 (2) × 10-18 J, where θD is the Debye temperature, Vo is the volume at T = 0, Ko' is the first derivative with respect to pressure of the incompressibility (Ko) and Y' is a Grüneisen parameter. The atomic displacement parameters were found to increase smoothly with T and could be fitted using Debye models with θD in the range 305-581 K. At 1223 K, the displacement of the F ions was found to be much less anisotropic than that in NaMgF3 at this temperature

    Pakistans ethnischer Flickenteppich und die Probleme der Verfassungsbildung

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    Die Seminararbeit setzt sich mit den Schwierigkeiten bei der Integration der verschiedenen ethnischen Gruppen innerhalb der pakistanischen Gesellschaft auseinander. Seit der Staatsgründung 1947 versucht die pakistanische Regierung gegen die vielfältigen separatistischen Strömungen und Bewegungen einzelner ethnischer Gruppen anzukämpfen. Welche ethnische Gruppen dabei mit separatistischen Strömungen die pakistanische Regierung beim Aufbau eines neuen Staates, der Formulierung gemeinsamer "policies" und einer Verfassung für die neue "Nation" in Schach hielten, ist Hauptgegenstand dieser Arbeit

    230Role of graft-facilitatory cells in human engraftment in the NOD-SCID mouse model

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    Scoping biological indicators of soil quality Phase II. Defra Final Contract Report SP0534

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    This report presents results from a field assessment of a limited suite of potential biological indicators of soil quality to investigate their suitability for national-scale soil monitoring

    Tidal turbines that survive?

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    Tidal turbines offer an exciting opportunity to exploit ocean current flows to generate sustainable energy. However, a key to their success is the ability to operate with minimal intervention in the ocean over extended periods (15-20 years). This talk explored the likely design and operational issues that will influence satisfactory performance associated with material corrosion and biofouling. The main difficulty is that turbine economic viability is capital driven so whole system, including operation and maintenance needs to be as cost-effective as possible. Although can use approaches developed from those applied for ship design and in the offshore industry there is a need to appreciate that cost-drivers are different. For instance a ‘Gold plated’ technology approach from oil and gas industry may not deliver cost-effective solutions

    Oligonucleotide-based therapy for FTD/ALS caused by the C9orf72 repeat expansion: a perspective

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    Amyotrophic lateral sclerosis (ALS) is a progressive and lethal disease of motor neuron degeneration, leading to paralysis of voluntary muscles and death by respiratory failure within five years of onset. Frontotemporal dementia (FTD) is characterised by degeneration of frontal and temporal lobes, leading to changes in personality, behaviour, and language, culminating in death within 5–10 years. Both of these diseases form a clinical, pathological, and genetic continuum of diseases, and this link has become clearer recently with the discovery of a hexanucleotide repeat expansion in the C9orf72 gene that causes the FTD/ALS spectrum, that is, c9FTD/ALS. Two basic mechanisms have been proposed as being potentially responsible for c9FTD/ALS: loss-of-function of the protein encoded by this gene (associated with aberrant DNA methylation) and gain of function through the formation of RNA foci or protein aggregates. These diseases currently lack any cure or effective treatment. Antisense oligonucleotides (ASOs) are modified nucleic acids that are able to silence targeted mRNAs or perform splice modulation, and the fact that they have proved efficient in repeat expansion diseases including myotonic dystrophy type 1 makes them ideal candidates for c9FTD/ALS therapy. Here, we discuss potential mechanisms and challenges for developing oligonucleotide-based therapy for c9FTD/ALS

    Optical properties of tungsten thin films perforated with a bidimensional array of subwavelength holes

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    We present a theorical investigation of the optical transmission of a dielectric grating carved in a tungsten layer. For appropriate wavelengths tungsten shows indeed a dielectric behaviour. Our numerical simulations leads to theoretical results similar to those found with metallic systems studied in earlier works. The interpretation of our results rests on the idea that the transmission is correlated with the resonant response of eigenmodes coupled to evanescent diffraction orders.Comment: 4 pages, 3 figure

    Peptide-conjugate antisense based splice-correction for Duchenne muscular dystrophy and other neuromuscular diseases

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    Duchenne muscular dystrophy (DMD) is an X-linked disorder characterized by progressive muscle degeneration, caused by the absence of dystrophin. Exon skipping by antisense oligonucleotides (ASOs) has recently gained recognition as therapeutic approach in DMD. Conjugation of a peptide to the phosphorodiamidate morpholino backbone (PMO) of ASOs generated the peptide-conjugated PMOs (PPMOs) that exhibit a dramatically improved pharmacokinetic profile. When tested in animal models, PPMOs demonstrate effective exon skipping in target muscles and prolonged duration of dystrophin restoration after a treatment regime. Herein we summarize the main pathophysiological features of DMD and the emergence of PPMOs as promising exon skipping agents aiming to rescue defective gene expression in DMD and other neuromuscular diseases. The listed PPMO laboratory findings correspond to latest trends in the field and highlight the obstacles that must be overcome prior to translating the animal-based research into clinical trials tailored to the needs of patients suffering from neuromuscular diseases
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