594 research outputs found
Controlling internal barrier in low loss BaTiO3 supercapacitors
Supercapacitor behavior has been reported in a number of oxides including reduced BaTiO3 ferroelectric ceramics. These so-called giant properties are however not easily controlled. We show here that the continuous coating of individual BaTiO3 grains by a silica shell in combination with spark plasma sintering is a way to process bulk composites having supercapacitor features with low dielectric losses and temperature stability. The silica shell acts both as an oxidation barrier during the processing and as a dielectric barrier in the final composite
Nucleotide trapping at the ATPase site of myosin subfragment 1 by a new interthiol crosslinking.
In Silico Scoring Of ALPL Gene Mutations Help To Distinguish Severe And Moderate Phenotypes In Hypophosphatasia
Heavy and light roles: myosin in the morphogenesis of the heart
Myosin is an essential component of cardiac muscle, from the onset of cardiogenesis through to the adult heart. Although traditionally known for its role in energy transduction and force development, recent studies
suggest that both myosin heavy-chain and myosin lightchain
proteins are required for a correctly formed heart.
Myosins are structural proteins that are not only expressed
from early stages of heart development, but when mutated
in humans they may give rise to congenital heart defects.
This review will discuss the roles of myosin, specifically
with regards to the developing heart. The expression of
each myosin protein will be described, and the effects that
altering expression has on the heart in embryogenesis in
different animal models will be discussed. The human
molecular genetics of the myosins will also be reviewed
Low loss coatings for the VIRGO large mirrors
présentée par L. PinardThe goal of the VIRGO program is to build a giant Michelson type interferometer (3 kilometer long arms) to detect gravitational waves. Large optical components (350 mm in diameter), having extremely low loss at 1064 nm, are needed. Today, the Ion beam Sputtering is the only deposition technique able to produce optical components with such performances. Consequently, a large ion beam sputtering deposition system was built to coat large optics up to 700 mm in diameter. The performances of this coater are described in term of layer uniformity on large scale and optical losses (absorption and scattering characterization). The VIRGO interferometer needs six main mirrors. The first set was ready in June 2002 and its installation is in progress on the VIRGO site (Italy). The optical performances of this first set are discussed. The requirements at 1064 nm are all satisfied. Indeed, the absorption level is close to 1 ppm (part per million), the scattering is lower than 5 ppm and the R.M.S. wavefront of these optics is lower than 8 nm on 150 mm in diameter. Finally, some solutions are proposed to further improve these performances, especially the absorption level (lower than 0.1 ppm) and the mechanical quality factor Q of the mirrors (thermal noise reduction)
Oro-Dental Features In Hypophosphatasia : A Valuable Phenotype For Disease Diagnosis And Evaluation Of Future Treatment Outcomes
Mild forms of hypophosphatasia mostly result from dominant negative effect of severe alleles or from compound heterozygosity for severe and moderate alleles
<p>Abstract</p> <p>Background</p> <p>Mild hypophosphatasia (HPP) phenotype may result from <it>ALPL </it>gene mutations exhibiting residual alkaline phosphatase activity or from severe heterozygous mutations exhibiting a dominant negative effect. In order to determine the cause of our failure to detect a second mutation by sequencing in patients with mild HPP and carrying on a single heterozygous mutation, we tested the possible dominant effect of 35 mutations carried by these patients.</p> <p>Methods</p> <p>We tested the mutations by site-directed mutagenesis. We also genotyped 8 exonic and intronic <it>ALPL </it>gene polymorphisms in the patients and in a control group in order to detect the possible existence of a recurrent intronic mild mutation.</p> <p>Results</p> <p>We found that most of the tested mutations exhibit a dominant negative effect that may account for the mild HPP phenotype, and that for at least some of the patients, a second mutation in linkage disequilibrium with a particular haplotype could not be ruled out.</p> <p>Conclusion</p> <p>Mild HPP results in part from compound heterozygosity for severe and moderate mutations, but also in a large part from heterozygous mutations with a dominant negative effect.</p
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