492 research outputs found
Variants Cause Spastic Paraplegia Associated with Cerebral Hypomyelination
Oculodentodigital dysplasia is an autosomal dominant disorder due to variants characterized by dysmorphic features. Neurologic symptoms have been described in some patients but without a clear neuroimaging pattern. To understand the pathophysiology underlying neurologic deficits in oculodentodigital dysplasia, we studied 8 consecutive patients presenting with hereditary spastic paraplegia due to variants. Clinical disease severity was highly variable. Cerebral MR imaging revealed variable white matter abnormalities, consistent with a hypomyelination pattern, and bilateral hypointense signal of the basal ganglia on T2-weighted images and/or magnetic susceptibility sequences, as seen in neurodegeneration with brain iron accumulation diseases. Patients with the more prominent basal ganglia abnormalities were the most disabled ones. This study suggests that -related hereditary spastic paraplegia is a complex neurodegenerative disease affecting both the myelin and the basal ganglia. variants should be considered in patients with hereditary spastic paraplegia presenting with brain hypomyelination, especially if associated with neurodegeneration and a brain iron accumulation pattern
How does fusion hindrance show up in medium-light systems? The case of 48Ca + 48Ca
The fusion excitation function of 48Ca + 48Ca has been measured above and well below the Coulomb barrier, thereby largely extending the energy range of a previous experiment down to very low cross sections. This system has a negative Q-value for compound nucleus formation. The fusion cross section decreases steadily below the barrier with no conspicuous change of slope below 300 μb. Coupled-channels calculations using a Woods–Saxon potential indicate that a large diffuseness parameter is needed to reproduce the sub-barrier cross sections. A close analogy with the case of 36S + 48Ca, with Q>0, is pointed out. The sign of the Q-value does not influence fusion cross sections down to the 300–600 nb leve
Selected nondigestible carbohydrates and prebiotics support the growth of probiotic fish bacteria mono-cultures in vitro
To search for nondigestible but fermentable (NDF) carbohydrates and prebiotics with a potency to promote the growth of selected bacteria in vitro.
The growth of three reference bacteria strains Bacillus subtilis LMG 7135(T), Carnobacterium piscicola LMG 9839, Lactobacillus plantarum LMG 9211 and one candidate probiotic bacteria Lactobacillus delbrueckii subsp. lactis was investigated over a minimum period of 48 h in the presence of beta-glucan, xylo-oligosaccharide, arabinoxylo-oligosaccharide, inulin, oligofructose and glucose. Besides the capability to grow on inulin and oligofructose containing media, a distinct high growth in beta-glucan based substrates and a low growth in (arabino)xylooligosaccharide containing media were evident for most bacteria tested. With the exception of B. subtilis and L. plantarum, other bacteria grew equally well or even better on different substrates than on glucose. The fermentation of studied carbohydrates by these micro-organisms was dominated by the production of acetic acid as the main short chain fatty acid.
Selected bacteria are able to ferment and grow on NDF and prebiotic carbohydrates but in a substrate dependent manner.
This study delivers a first screening of which NDF or prebiotic carbohydrates are the most promising for aquaculture feed supplementations
Restricted Application of Insecticides: A Promising Tsetse Control Technique, but What Do the Farmers Think of It?
Restricted application of insecticides to cattle is a cheap and safe farmer-based method to control tsetse and the diseases they transmit, i.e. human and animal African trypanosomoses. The efficiency of this new control method has been demonstrated earlier but no data is available on its perception and adoption intensity by farmers. We studied these two features in Burkina Faso, where the method has diffused thanks to two development projects. The study allowed identifying three groups of farmers with various adoption intensities, of which one was modern and two traditional. The economic benefit and the farmers' knowledge of the epidemiological system appeared to have a low impact on the early adoption process whereas some modern practices, as well as social factors appeared critical. The quality of technical support provided to the farmers had also a great influence on the adoption rate. The study highlighted individual variations in risk perceptions and benefits, as well as the prominent role of the socio-technical network of cattle farmers. The results of the study are discussed to highlight the factors that should be taken into consideration, to move discoveries from bench to field for an improved control of trypanosomoses vectors
Deformation of the N=Z nucleus 76Sr using beta-decay studies
A novel method of deducing the deformation of the N=Z nucleus 76Sr is
presented. It is based on the comparison of the experimental Gamow-Teller
strength distribution B(GT) from its beta decay with the results of QRPA
calculations. This method confirms previous indications of the strong prolate
deformation of this nucleus in a totally independent way. The measurement has
been carried out with a large Total Absorption gamma Spectrometer, "Lucrecia",
newly installed at CERN-ISOLDE.Comment: Accepted in Phys. Rev. Letter
Binary reaction decays from 24Mg+12C
Charged particle and gamma decays in 24Mg* are investigated for excitation
energies where quasimolecular resonances appear in 12C+12C collisions. Various
theoretical predictions for the occurence of superdeformed and hyperdeformed
bands associated with resonance structures with low spin are discussed within
the measured 24Mg* excitation energy region. The inverse kinematics reaction
24Mg+12C is studied at E_lab(24Mg) = 130 MeV, an energy which enables the
population of 24Mg states decaying into 12C+12C resonant break-up states.
Exclusive data were collected with the Binary Reaction Spectrometer in
coincidence with EUROBALL IV installed at the VIVITRON Tandem facility at
Strasbourg. Specific structures with large deformation were selectively
populated in binary reactions and their associated gamma decays studied.
Coincident events associated with inelastic and alpha-transfer channels have
been selected by choosing the excitation energy or the entry point via the
two-body Q-values. The analysis of the binary reaction channels is presented
with a particular emphasis on 24Mg-gamma, 20Ne-gamma and 16O-gamma
coincidences. New information (spin and branching ratios) is deduced on
high-energy states in 24Mg and 16O, respectively.Comment: 27 pages, 8 figures, 1 tabl
Reaction mechanisms in 24Mg+12C and 32S+24Mg
The occurence of "exotic" shapes in light N=Z alpha-like nuclei is
investigated for 24Mg+12C and 32S+24Mg. Various approaches of superdeformed and
hyperdeformed bands associated with quasimolecular resonant structures with low
spin are presented. For both reactions, exclusive data were collected with the
Binary Reaction Spectrometer in coincidence with EUROBALL IV installed at the
VIVITRON Tandem facility of Strasbourg. Specific structures with large
deformation were selectively populated in binary reactions and their associated
-decays studied. The analysis of the binary and ternary reaction
channels is discussed.Comment: 7 pages, 4 figures, Paper presented at the Fusion08 International
Conference on New Aspects of Heavy Ion Collisions Near the Coulomb Barrier,
Chicago. Proceedings to be published by AIP Conference Proceedings Illinois,
USA, September 22-26, 200
Search for highly excited states in 28Si
The theoretical and experimental determination of superdeformed states in nuclei in the mass region A≤40 has been since a long time one of the major challenges of nuclear structure studies. Despite the considerable experimental and theoretical work dedicated to this topic, up to now superdeformed bands have been found in only two nuclei, 36Ar and 40Ca. While the experimental signature of the superdeformed nature of those states is irrefutable, their theoretical interpretation is still uncertain. In particular, it is not clear whether clusterisation is responsible of the onset of superdeformation. For this reason, we wanted to investigate an even lighter system, 28Si, where a number of theoretical calculations predict the presence of superdeformation as an effect of the cluster structure of the nucleus
Clusters in Light Nuclei
A great deal of research work has been undertaken in the alpha-clustering
study since the pioneering discovery, half a century ago, of 12C+12C molecular
resonances. Our knowledge of the field of the physics of nuclear molecules has
increased considerably and nuclear clustering remains one of the most fruitful
domains of nuclear physics, facing some of the greatest challenges and
opportunities in the years ahead. In this work, the occurence of "exotic"
shapes in light N=Z alpha-like nuclei is investigated. Various approaches of
superdeformed and hyperdeformed bands associated with quasimolecular resonant
structures are presented. Results on clustering aspects are also discussed for
light neutron-rich Oxygen isotopes.Comment: 12 pages, 5 figures. Invited Talk presented by C. Beck at the
Zakopane Conference on Nuclear Physics "Extremes of the Nuclear Landscape"
XLV in the series of Zakopane Schools of Physics - International Symposium -
Zakopane, Poland, August 30 - September 5, 2010.To be publihed in Acta
Physica Polonica B42 no 3, March 201
Fusion reactions of 58,64Ni+124Sn
International audience; In order to better understand the influence of transfer in sub-barrier nuclear reactions, cross sections for the system ^58,64Ni+^124Sn have been measured down to 0.5-1 mub and compared to detailed coupledchannel calculations. In agreement with a phenomenological Q-value systematics, calculations show the importance of including the coupling to the transfer channel for these heavy systems. No clear evidence of fusion hindrance is observed, probably due to the fact that the cross sections measured in this experiment are not low enough for the appearance of that phenomenon
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