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Motor nerve excitability studies by "threshold tracking" in amyotrophic lateral sclerosis (ALS) revealed heterogeneous abnormalities in motor axon membrane function possibly depending on disease stage. It remains unclear to which extent the excitability deviations reflect a pathogenic mechanism in ALS or are merely a consequence of axonal degeneration. We investigated motor axon excitability in presymptomatic and symptomatic SOD1(G127X) mutants, a mouse model of ALS with late clinical onset and rapid disease progression. After clinical onset, there was a rapid loss of functional motor units associated with an increase in rheobase and strength-duration time constant, an increase in refractoriness at the expense of the superexcitability, larger than normal threshold deviations during both depolarizing and hyperpolarizing threshold electrotonus with impaired accommodation and reduction of the input conductance. These abnormalities progressed rapidly over a few days and were associated with morphological evidence of ongoing axonal degeneration. Presymptomatic mice with unaltered motor performance at rotor-rod measurement also had an increase in refractoriness at the expense of the superexcitability during the recovery cycle. This was, however, associated with smaller than normal deviations during threshold electrotonus, and a steeper resting current-threshold slope indicating slight axonal depolarization in agreement with motoneuronal hyperexcitability indicated by enhanced F-waves. Our data suggest that SOD1(G127X) motor axons undergo a state of membrane depolarization; however, during rapid motoneuron loss disease-specific nerve excitability measures are confounded by excitability changes in degenerating but still conducting axons. These findings should be considered in the interpretation of disease-stage-related nerve excitability changes in ALS.

Item does not contain fulltext, Background and study aims: The clinical utility of narrow-band imaging (NBI) for Barrett's esophagus is limited by the multiplicity of classification schemes. We evaluated the interobserver agreement and accuracy of a new consensus-driven simplified binary classification of NBI surface patterns.

Myelin protein zero mutations were found to produce Charcot–Marie–Tooth disease phenotypes with various degrees of myelin impairment and axonal loss, ranging from the mild ‘demyelinating’ adult form to severe and early onset forms. Protein zero deficient homozygous mice () show a severe and progressive dysmyelinating neuropathy from birth with compromised myelin compaction, hypomyelination and distal axonal degeneration. A previous study using immunofluorescence showed that motor nerves deficient of myelin protein zero upregulate the NaV1.8 voltage gated sodium channel isoform, which is normally present only in restricted populations of sensory axons. The aim of this study was to investigate the function of motor axons in protein zero-deficient mice with particular emphasis on ectopic NaV1.8 voltage gated sodium channel. We combined ‘threshold tracking’ excitability studies with conventional nerve conduction studies, behavioural studies using rotor-rod measurements, and histological measures to assess membrane dysfunction and its progression in protein zero deficient homozygous mutants as compared with age-matched wild-type controls. The involvement of NaV1.8 was investigated by pharmacologic block using the subtype-selective NaV1.8 blocker A-803467 and chronically in NaV1.8 knock-outs. We found that in the context of dysmyelination, abnormal potassium ion currents and membrane depolarization, the ectopic NaV1.8 channels further impair the motor axon excitability in protein zero deficient homozygous mutants to an extent that precipitates conduction failure in severely affected axons. Our data suggest that a NaV1.8 channelopathy contributed to the poor motor function of protein zero deficient homozygous mutants, and that the conduction failure was associated with partially reversible reduction of the electrically evoked muscle response and of the clinical function as indicated by the partial recovery of function at rotor-rod measurements. As a consequence of these fi, Myelin protein zero mutations were found to produce Charcot-Marie-Tooth disease phenotypes with various degrees of myelin impairment and axonal loss, ranging from the mild 'demyelinating' adult form to severe and early onset forms. Protein zero deficient homozygous mice ( ) show a severe and progressive dysmyelinating neuropathy from birth with compromised myelin compaction, hypomyelination and distal axonal degeneration. A previous study using immunofluorescence showed that motor nerves deficient of myelin protein zero upregulate the Na(V)1.8 voltage gated sodium channel isoform, which is normally present only in restricted populations of sensory axons. The aim of this study was to investigate the function of motor axons in protein zero-deficient mice with particular emphasis on ectopic Na(V)1.8 voltage gated sodium channel. We combined 'threshold tracking' excitability studies with conventional nerve conduction studies, behavioural studies using rotor-rod measurements, and histological measures to assess membrane dysfunction and its progression in protein zero deficient homozygous mutants as compared with age-matched wild-type controls. The involvement of Na(V)1.8 was investigated by pharmacologic block using the subtype-selective Na(V)1.8 blocker A-803467 and chronically in Na(V)1.8 knock-outs. We found that in the context of dysmyelination, abnormal potassium ion currents and membrane depolarization, the ectopic Na(V)1.8 channels further impair the motor axon excitability in protein zero deficient homozygous mutants to an extent that precipitates conduction failure in severely affected axons. Our data suggest that a Na(V)1.8 channelopathy contributed to the poor motor function of protein zero deficient homozygous mutants, and that the conduction failure was associated with partially reversible reduction of the electrically evoked muscle response and of the clinical function as indicated by the partial recovery of function at rotor-rod measurements. As a conseque

This paper describes the wave-front correction system developed for the Sunrise balloon telescope, and provides information about its in-flight performance. For the correction of low-order aberrations, a Correlating Wave-Front Sensor (CWS) was used. It consisted of a six-element Shack-Hartmann wave-front sensor (WFS), a fast tip-tilt mirror for the compensation of image motion, and an active telescope secondary mirror for focus correction. The CWS delivered a stabilized image with a precision of 0.04 arcsec (rms), whenever the coarse pointing was better than 90 arcsec peak-to-peak. The automatic focus adjustment maintained a focus stability of 0.01 waves in the focal plane of the CWS. During the 5.5 day flight, good image quality and stability was achieved during 33 hours, containing 45 sequences that lasted between 10 and 45 minutes.

The Imaging Magnetograph eXperiment (IMaX) is a spectropolarimeter built by four institutions in Spain that flew on board the Sunrise balloon-borne telesocope in June 2009 for almost six days over the Arctic Circle. As a polarimeter IMaX uses fast polarization modulation (based on the use of two liquid crystal retarders), real-time image accumulation, and dual beam polarimetry to reach polarization sensitivities of 0.1%. As a spectrograph, the instrument uses a LiNbO3 etalon in double pass and a narrow band pre-filter to achieve a spectral resolution of 85 mAA. IMaX uses the high Zeeman sensitive line of Fe I at 5250.2 AA and observes all four Stokes parameters at various points inside the spectral line. This allows vector magnetograms, Dopplergrams, and intensity frames to be produced that, after reconstruction, reach spatial resolutions in the 0.15-0.18 arcsec range over a 50x50 arcsec FOV. Time cadences vary between ten and 33 seconds, although the shortest one only includes longitudinal polarimetry. The spectral line is sampled in various ways depending on the applied observing mode, from just two points inside the line to 11 of them. All observing modes include one extra wavelength point in the nearby continuum. Gauss equivalent sensitivities are four Gauss for longitudinal fields and 80 Gauss for transverse fields per wavelength sample. The LOS velocities are estimated with statistical errors of the order of 5-40 m/s. The design, calibration and integration phases of the instrument, together with the implemented data reduction scheme are described in some detail., Comment: 17 figures

This work reports about the preparation of plasma polymerized thin films of perylene with thicknesses ̃30-150 nm and their characterization by different methods and the analysis of their optical properties. Highly absorbent and fluorescent films have been obtained by this method that combines the sublimation of the perylene molecules and their controlled polymerization by the interaction with remote Ar plasma. The polymeric films are very flat with a root mean square (rms) roughness in the range 0.3-0.4 nm. In contrast with the sublimated layers of perylene that present a high scattering of light, the polymerized films depict the well-defined absorption bands in the region 400-450 nm and fluorescence spectra of the perylene molecule at ̃475 nm. The films are formed by a matrix formed by cross-linked fragments of perylene and intact molecules that confer the observed optical properties to this material. The optical and microstructural characteristics of this type of thin films and the possibility to perform their deposition by using lithographic procedures make them suitable for their integration into photonic components for various applications. A preliminary study of the use of these films as an optical sensor of NO2 is also presented. © 2009 American Chemical Society.

Mechanically stable and insoluble fluorescent thin films have been deposited by sublimating Rhodamine 6G laser dye in the downstream region of a low-power microwave ECR plasma using an experimental set-up designed to control the interaction of the dye molecule with the glow discharge. The use of reactive organosilane plasmas allows to control the dye distribution inside the matrix, leading to solid nanocomposite thin films containing non-aggregated dye molecules. The suppression of aggregates is a key issue to avoid fluorescence quenching. The obtained nanocomposite films are interesting because of their strong absorption and high fluorescence emission. In addition, they can be patterned using in situ plasma treatments in order to produce optically functional devices. A figure is presented. ©2009 Wiley-VCH Verlag GmbH & Co. KGaA, Weinheim.

Solgel phenyl functionalized silica thin films were used as a host matrix to embed photochromic molecules (1,3,3,5,6-pentamethyl-spiro[indoline-2-3′-[quinolino]oxazinel, 3-(2,4-dimethoxyphenyl)-3-(4-methoxyphenyl)-3H-naphtho[2,1-b]pyran and 3,3-diphenyl-3H-naphtho[2,1-b]pyran). The coatings were doped with individual dyes and mixtures of them. The optical properties of the colored and colorless forms of these photochromic materials were studied using variable angle spectroscopic ellipsometry. Light polarization analyses allowed an exhaustive ellipsometric characterization of these materials. Important changes were obtained in the refractive indices of the colored and colorless samples, with differences of up to 0.012 and 0.018 in the real and imaginary components, respectively. Good correlation was observed on the optical parameters of samples doped with individual dyes and mixtures of them. Standard transmission measurements were used to study the dynamic photochromic transitions and obtain the kinetic constants. The high capabilities of ellipsometry for the high-accuracy characterization of dynamic photochromic transitions were demonstrated, This technique allowed measuring variations in the populations of colored and colorless molecules, resulting from variations of temperature lower than ±0.1°C. © 2007 Optical Society of America.