Murine model of autosomal dominant retinitis pigmentosa generated by targeted deletion at codon 307 of the rds-peripherin gene

We introduced a targeted single base deletion at codon 307 of the rds-peripherin gene in mice, similar mutations being known to cause autosomal dominant retinitis pigmentosa (RP) in man. Histopathological and electroretinographic analysis indicate that the retinopathy in mice homozygous for the codon 307 mutation appears more rapid than that in the naturally occurring null mutant, the rds(-/-) mouse, suggesting that the rds-307 mutation displays a dominant negative phenotype in combination with that due to haplosufficiency. RP is the most prevalent cause of registered visual handicap in those of working age in developed countries, the 50 or so mutations so far identified within the RDS-peripherin gene accounting for up to 10% of dominant cases of the disease. Given the sequence homologies that exist between the murine rds-peripherin and the human RDS-peripherin gene, this disease model, the first to be generated for peripherin-based RP using gene targeting techniques, should in principle be of value in the work-up in mice of therapeutics capable of targeting transcripts derived from the human gene.

Mushroom: An Oracular Sound Processor

Composers of digital music today have a bewildering variety of sound-processing tools and techniques at their disposal. At their best, these tools allow composers to hone a sound to perfection. However, they can also lead us into a routine which bypasses avenues of experimentation, simply because the known tools work so well and their sonic output is so attractive. An alternative strategy is oracular sound processing. An oracular sound processor creates a derived version of its input whose characteristics could not have been fully predicted, while affording the user little or no parametric control over the process.

A comparison of the strengths of gastropod shells with forces generated by potential crab predators

Carcinus manenas, Liocarcinus puber and Cancer pagurs are thought to be three likely crab predators of the gastropod Calliostoma Zizyphinum. In order to compare the strenghts of predators and their prey, the whole shell and aperture lip strengh of white and pink Calliostoma morphotypes and the maximum forces exerted by the chelipeds of three crab species were measured. Although white shells were thicker than pink shells, Calliostoma colour morphotyes did not differ significantly in either the force required to break the shell lip or the whole shell. Both Liocarcinus puber and Carcinus maenas have dimorphic chelipeds and their “crusher” chelipeds deliver almost double the forces generated by the‘cutter’chelipeds. In constrast, Cancer pagurus has monomorphic chelipeds both delivering similar forces. When compared with Calliostoma shell strenght, the forces generated by the‘crusher’chelipeds of most L. puber tested were, in general, sufficient to break the shell lip of Calliostoma shells, whereas forces generated by the‘cutter’chelipeds of only the larger individuals were sufficient to break the shell lip. In C. manenas, forces generated by both the‘cutter’and‘crusher’chelipeds often exceeded the minimum recorded force required to break the shell lip and the‘crusher’cheliped reached the minimum force required to break whole Calliostoma shells. Both chelipeds of all C. pagurus tested generated forces in excess of the minimum required to break the shell lip, and the proportion of individuals capable of generating the minimum force required to break the whole shell increased with the size of the size of the crab. Carcinus maenas and Cancer pagurus were capable of breaking both the shell lips and the whole shells of a wider range of shell sizes than L. puber.

Investigation on localisation accuracy for first and higher order ambisonics reproduced sound sources

Ambisonics and higher order ambisonics (HOA) technologies aim at reproducing sound field either synthesised or previously recorded with dedicated microphones. Based on a spherical harmonic decomposition, the sound field is more precisely described when higher-order components are used. The presented study evaluated the perceptual and objective localisation accuracy of the sound field encoded with four microphones of order one to four and decoded over a ring of loudspeakers. A perceptual test showed an improvement of the localisation with higher order ambisonic microphones. Reproduced localisation indices were estimated for the four microphones and the respective synthetic systems of order one to four. The perceptual and objective analysis revealed the same conclusions. The localisation accuracy depends on the ambisonic order as well as the source incidence. Furthermore, impairments linked to the microphones were highlighted.

Expertise is perceived from both sound and body movement in musical performance

Music is a rich form of nonverbal communication, in which the movements that expert musicians make during performance can influence the perception of expressive and structural features of the music. Whether the actual skill of a musician is perceivable from vision of movement was examined. In Experiment 1, musicians and non-musicians rated performances by novice, intermediate and expert clarinettists from point-light animations of their movements, sound recordings, or both. Performances by clarinettists of more advanced skill level were rated significantly higher from vision of movements, although this effect was stronger when sound was also presented. In Experiment 2, movements and sound from the novice and expert clarinettists' performances were switched for half the presentations, and were matched for the rest. Ratings of novice music were significantly higher when presented with expert movements, although the opposite was not found for expert sound presented with novice movements. No perceptual effect of raters' own level of musicianship was found in either experiment. These results suggest that expertise is perceivable from vision of musicians' body movements, although perception of skill from sound is dominant. The results from Experiment 2 further indicate a cross-modal effect of vision and audition on the perception of musical expertise. © 2012 Elsevier B.V.

The Origin of Type I Spicule Oscillations

We use images of high spatial and temporal resolution, obtained with the Rapid Oscillations in the Solar Atmosphere instrument at the Dunn Solar Telescope, to reveal how the generation of transverse waves in Type I spicules is a direct result of longitudinal oscillations occurring in the photosphere. Here we show how pressure oscillations, with periodicities in the range of 130–440 s, manifest in small-scale photospheric magnetic bright points, and generate kink waves in the Sun’s outer atmosphere with transverse velocities approaching the local sound speed. Through comparison of our observations with advanced two-dimensional magnetohydrodynamic simulations, we provide evidence for how magnetoacoustic oscillations, generated at the solar surface, funnel upward along Type I spicule structures, before undergoing longitudinal-to-transverse mode conversion into waves at twice the initial driving frequency. The resulting kink modes are visible in chromospheric plasma, with periodicities of 65–220 s, and amplitudes often exceeding 400 km. A sausage mode oscillation also arises as a consequence of the photospheric driver, which is visible in both simulated and observational time series. We conclude that the mode conversion and period modi?cation is a direct consequence of the 90? phase shift encompassing opposite sides of the photospheric driver. The chromospheric energy ?ux of these waves are estimated to be ˜3 × 105 W m-2, which indicates that they are suf?ciently energetic to accelerate the solar wind and heat the localized corona to its multi-million degree temperatures.