Search results for "EMMA"
showing 10 items of 629 documents
Inventaire de la collection Emmanuel Guyot. Musée archéologique, Dijon: (Saisie et mise en page ; réalisation de l'index)
1996
Inventaire réalisé par D. Périchon, Musée archéologique, Dijon.; 100 p. ; fig. Index.
Homozygous mutations incaveolin-3cause a severe form of rippling muscle disease
2003
Heterozygous missense mutations in the caveolin-3 gene (CAV3) cause different muscle disorders. Most patients with CAV3 alterations present with rippling muscle disease (RMD) characterized by signs of increased muscle irritability without muscle weakness. In some patients, CAV3 mutations underlie the progressive limb-girdle muscular dystrophy type 1C (LGMD1C). Here, we report two unrelated patients with novel homozygous mutations (L86P and A92T) in CAV3. Both presented with a more severe clinical phenotype than usually seen in RMD. Immunohistochemical and immunoblot analyses of muscle biopsies showed a strong reduction of caveolin-3 in both homozygous RMD patients similar to the findings in…
DNA-fragmentation and expression of apoptosis-related proteins in muscular dystrophies
1997
Although numerous sarcolemmal protein defects in muscular dystrophies have been identified, the mechanisms linking these defects and muscle fibre degeneration are not fully characterized. As there is evidence that apoptosis is part of muscle fibre loss in dystrophin-deficient mdx-mice, apoptotic muscle fibre death may also play a role in humans with muscular dystrophies. We investigated in-situ DNA-fragmentation by the TUNEL-method and expression of apoptosis-related proteins immunohistochemically in 14 children suffering from deficiencies of dystrophin, adhalin, and merosin, and found TUNEL-positive chromatin-cleavage of muscle fibre nuclei in about 10% of non-necrotic muscle fibres. DNA-f…
Data Backup Dilemma
2016
When the Great East Japan Earthquake struck in 2011, several municipalities lost their residential data including backup. Since none of them had ever considered the total loss of data, data backup policy had been paid little attention. In many cases, the backup tapes were simply stored inside the server room, just beside the server rack. Following the calamity, the Japanese national government tried to introduce a data backup system to municipalities using the cloud. The purpose was to secure the safekeeping of backup data. However, municipalities were reluctant to go along with this since overcoming the loss of network connectivity during an earthquake remained foremost in their minds. The…
On Fibrations Between Internal Groupoids and Their Normalizations
2018
We characterize fibrations and $$*$$ -fibrations in the 2-category of internal groupoids in terms of the comparison functor from certain pullbacks to the corresponding strong homotopy pullbacks. As an application, we deduce the internal version of the Brown exact sequence for $$*$$ -fibrations from the internal version of the Gabriel–Zisman exact sequence. We also analyse fibrations and $$*$$ -fibrations in the category of arrows and study when the normalization functor preserves and reflects them. This analysis allows us to give a characterization of protomodular categories using strong homotopy kernels and a generalization of the Snake Lemma.
High intensity neutrino oscillation facilities in Europe
2013
The EUROnu project has studied three possible options for future, high intensity neutrino oscillation facilities in Europe. The first is a Super Beam, in which the neutrinos come from the decay of pions created by bombarding targets with a 4 MW proton beam from the CERN High Power Superconducting Proton Linac. The far detector for this facility is the 500 kt MEMPHYS water Cherenkov, located in the Frejus tunnel. The second facility is the Neutrino Factory, in which the neutrinos come from the decay of mu(+) and mu(-) beams in a storage ring. The far detector in this case is a 100 kt magnetized iron neutrino detector at a baseline of 2000 km. The third option is a Beta Beam, in which the neu…
Step-by-step integration for fractional operators
2018
Abstract In this paper, an approach based on the definition of the Riemann–Liouville fractional operators is proposed in order to provide a different discretisation technique as alternative to the Grunwald–Letnikov operators. The proposed Riemann–Liouville discretisation consists of performing step-by-step integration based upon the discretisation of the function f(t). It has been shown that, as f(t) is discretised as stepwise or piecewise function, the Riemann–Liouville fractional integral and derivative are governing by operators very similar to the Grunwald–Letnikov operators. In order to show the accuracy and capabilities of the proposed Riemann–Liouville discretisation technique and th…
germline mutations in women with familial breast cancer and a relative with haematological malignancy
2009
Biallelic inactivation of the ATM gene causes ataxia-telangiectasia (A-T), a complex neurological disease associated with a high risk of leukaemias and lymphomas. Mothers of A-T children, obligate ATM heterozygote mutation carriers, have a breast cancer (BC) relative risk of about 3. The frequency of ATM carriers in BC women with a BC family history has been estimated to be 2.70%. To further our clinical understanding of familial BC and examine whether haematological malignancies are predictive of ATM germline mutation, we estimated the frequency of heterozygote mutation carriers in a series of 122 BC women with a family history of both BC and haematological malignancy and without BRCA1/2 m…
More planet and less profit? The ethical dilemma of an oil producing nation
2019
Every oil producing nation is confronted with a complex and fundamental ethical dilemma. On the one hand, there are moral arguments for the nation to use the petroleum resource for the benefit of society and make it available for countries who do not have this natural resource endowment. On the other hand, there are moral arguments for not extracting and using fossil fuels because of CO2 emissions. In short, this creates tension between the need for government revenues to finance welfare benefits and the objective of preserving the environment. A complicating factor is that a nation's domestic oil and gas activities are in its nature global because the activities have a direct impact on the…
Development and validation of the Brief Parental Burnout Scale (BPBS).
2021
Since parental burnout can have detrimental consequences for parents, couples, and children, easy-to-use, reliable, and valid practical tools for the early detection of parental burnout are required. We developed and validated a brief scale to identify burned-out parents and parents at burnout risk. In Study 1, we developed the 5-item Brief Parental Burnout scale (BPBs) based on the gold standard measure of parental burnout, the Parental Burnout Assessment (PBA). We applied Item Response Theory Graded Response Model (GRM) analyses on the data of 1,725 Finnish parents. Compared against the PBA total score, the five selected items showed both high sensitivity and specificity in screening pare…