Correlation between left ventricular hypertrophy and GAA trinucleotide repeat length in Friedreich's ataxia

BACKGROUND: Friedreich's ataxia (FA), the most common inherited ataxia, is associated frequently with cardiac hypertrophy, and death is often cardiac related. Recently, the disease has been associated with a mutation that consists of an unstable expansion of GAA repeats in the first intron of the gene encoding frataxin on chromosome 9. METHODS AND RESULTS: We studied 44 consecutive patients with FA, determined the size of GAA expansions in the frataxin gene, and examined the relation between the genotype and cardiac phenotype assessed by M-mode and two-dimensional echocardiography. All the patients were homozygous for the mutation. The size of the GAA expansion on the smaller allele varied from 270 to 1200. We found a correlation between the size of GAA expansion and the left ventricular wall thickness (r = .51, P < .001) and the left ventricular mass index (r = .45, P = .002). Left ventricular hypertrophy was observed in 81% of patients with a number of GAA repeats above the median value of 770 compared with only 14% in the other group (P = .002). CONCLUSIONS: These data demonstrate that in FA, the severity of left ventricular hypertrophy is related to the number of GAA repeats. These results suggest that abnormalities of the gene encoding frataxin, a protein of unknown function highly expressed in the normal heart, may play an important role in the modulation of cardiac hypertrophy.     

Infrared Detection of Hot Spots in Energized Electrical Equipment

An important aspect of plant operation is preventive maintenance of electrical apparatus. Local thermal overloads or bad electrical connections often appear as localized hot spots in energized electrical equipment. A survey of the use of infrared detection systems for location of such hot spots is presented. A tutorial introduction to infrared detection and imaging systems is given with emphasis on the requirements imposed by this particular application. Following this, a survey of commercially available systems suitable for remote detection of hot spots is presented. Finally, the highlights of new developments in infrared technology are discussed.     

Preparation of transparent PMMA/Fe(IO3)3 nanocomposite films from microemulsion polymerization

Polymethyl methacrylate (PMMA)/Fe(IO₃)₃ nanocomposite thin films are obtained by in situ particle generation in microemulsions and subsequent photopolymerization of a mixture containing methyl methacrylate, trimethylolpropane triacrylate, and crystallized iron iodate (Fe(IO₃)₃) nanorods. Hyper‐Rayleigh scattering measurements combined with X‐ray diffraction, transmission electron microscopy, and dynamic light scattering are first used to probe in situ the crystallization kinetics of iron iodate nanorods in water‐in‐oil microemulsions prepared with methyl methacrylate as the oil phase and marlophen NP12 as a surfactant. Trimethylolpropane triacrylate is then added as a crosslinker before spin‐coating. Films are deposited on glass substrates for the nonlinear optical characterizations and on silicon wafers for the piezoelectric and mechanical measurements. Nanocomposite films treated by corona discharge are finally characterized through optical microscopy, laser Doppler vibrometry, and Brillouin spectroscopy. © 2013 Wiley Periodicals, Inc. J. Appl. Polym. Sci. 130: 1203‐1211, 2013     

Nanocomposite piezoelectric films of P(VDF‐TrFE)/LiNbO3

Piezoelectric films were prepared by incorporation of lithium niobate (LiNbO₃) nanoparticles into copolymer of vinylidene difluoride and trifluoroethylene. Nanoparticles of LiNbO₃ with ferroelectric phase were successfully synthesized and dispersed homogenously by ultrasonication in the copolymer matrix without any surfactant or surface functionalization. The nanocomposites were fully characterized by electronic microscopy, X‐ray diffraction, differential scanning calorimetry, dynamical mechanical analysis, and piezometer. Surprisingly, the copolymer matrix crystallinity and morphology were not affected by the incorporation of nanoparticles. Therefore the nanocomposites remained good mechanicals properties and high ferroelectricity coupled to nonlinear optical activity thanks to the noncentro symmetric space group of lithium niobate. This could be a novel approach to develop new multifunctional materials. © 2012 Wiley Periodicals, Inc. J. Appl. Polym. Sci., 2013     

Multivalent Interactions between an Aromatic Helical Foldamer and a DNA G‐Quadruplex in the Solid State

Quinoline‐based oligoamide foldamers have been identified as a potent class of ligands for G‐quadruplex DNA. Their helical structure is thought to target G‐quadruplex loops or grooves and not G‐tetrads. We report a co‐crystal structure of the antiparallel hairpin dimeric DNA G‐quadruplex (G₄T₄G₄)₂ with tetramer 1 —a helically folded oligo‐quinolinecarboxamide bearing cationic side chains—that is consistent with this hypothesis. Multivalent foldamer–DNA interactions that modify the packing of (G₄T₄G₄)₂ in the solid state are observed.     

Influence of the microstructure of gums on the mechanical properties of silicone high consistency rubbers

This paper is devoted to the characterization and processing of high molar mass vinyl‐bearing polysiloxanes in high consistency silicone rubber (HCR) formulations. The molar masses of five different polydimethylsiloxane gums, bearing vinyl groups at the ends and along their chains, were evaluated by size exclusion chromatography and rheometry. ²⁹Si and ¹H NMR spectroscopy allowed the precise determination of the vinyl content and of the distribution in the different polymers. Typical HCRs formulated from these gums were heat‐cured to process silicone rubber materials that were then tested mechanically. The macromolecular properties were correlated to the final material network structure. The amount of reactive vinyl moieties, rather than their distribution along or at the end of chains, is a key parameter to tailor the material mechanical properties. © 2016 Society of Chemical Industry     

Productivity of yam-based systems with herbaceous legumes and short fallows in the Guinea-Sudan transition zone of Benin

The principal driving force in agricultural research is to increase the yield of food crops. For farming to remain productive, it will be necessary to replenish the nutrients removed or lost from the soil. The objective of this study was to determine the impact of yam-based systems on soil productivity (dry matter production, nutrients recycled or removed, profitability and soil fertility changes). We compared smallholders’ traditional systems (1-year fallow of Andropogonon gayanus -yam rotation; maize-yam rotation) with yam-based systems with legumes (intercropped Aeschynomene histrix with maize-yam rotation; intercropped Mucuna pruriens with maize-yam rotation). The production of dry matter (tubers, shoots), nutrients removed or recycled, and soil properties were significantly improved on yam-based systems with legumes in comparison with traditional systems. Year × Treatment interactions influenced significantly the tuber dry matter production. Site × Treatment and Treatment × Farmer interactions affected significantly nutrients removed or recycled. The amount of nutrients recycled or removed was dependent on the dry matter production that, in turn, depended on soil fertility, rainfall and farmers’ effect.Yam-based systems with legumes brought a higher present value than traditional systems in the first 4 years and appeared attractive for land, labour and cash productivities.     

Optimal control law for classical and multiconjugate adaptive optics

Classical adaptive optics (AO) is now a widespread technique for high-resolution imaging with astronomical ground-based telescopes. It generally uses simple and efficient control algorithms. Multiconjugate adaptive optics (MCAO) is a more recent and very promising technique that should extend the corrected field of view. This technique has not yet been experimentally validated, but simulations already show its high potential. The importance for MCAO of an optimal reconstruction using turbulence spatial statistics has already been demonstrated through open-loop simulations. We propose an optimal closed-loop control law that accounts for both spatial and temporal statistics. The prior information on the turbulence, as well as on the wave-front sensing noise, is expressed in a state-space model. The optimal phase estimation is then given by a Kalman filter. The equations describing the system are given and the underlying assumptions explained. The control law is then derived. The gain brought by this approach is demonstrated through MCAO numerical simulations representative of astronomical observation on a 8-m-class telescope in the near infrared. We also discuss the application of this control approach to classical AO. Even in classical AO, the technique could be relevant especially for future extreme AO systems.     

On the designing of densely dispersion-managed optical fiber systems for ultrafast optical communication

We present some theoretical and experimental results which suggest the possibility of constructing a non-empirical methodology of designing optical transmission systems with ultra high bit-rate per channel. Theoretically, we present an average dispersion decreasing densely dispersion-managed (A4dm) fiber system, which exhibits many advantages over the densely dispersion-managed fiber system, such as the possibility of transmitting chirp-free Gaussian pulses at 160 Gbit/s per channel over transoceanic distances, with a reduced energy and minimal intra-channel interaction. Experimentally we present generation of a 160-GHz picosecond pulse train at 1550 nm using multiple four-wave mixing temporal compression of an initial dual frequency beat signal in the anomalous-dispersion regime of a non-zero dispersion shifted fiber. A complete intensity and phase characterization of the pulse train by means of a frequency-resolved optical gating technique is achieved, showing generation of transform-limited pedestal-free Gaussian pulses.