Usher Syndrome in the Inner Ear: Etiologies and Advances in Gene Therapy

Hearing loss is the most common sensory disorder with ~466 million people worldwide affected, representing about 5% of the population. A substantial portion of hearing loss is genetic. Hearing loss can either be non-syndromic, if hearing loss is the only clinical manifestation, or syndromic, if the hearing loss is accompanied by a collage of other clinical manifestations. Usher syndrome is a syndromic form of genetic hearing loss that is accompanied by impaired vision associated with retinitis pigmentosa and, in many cases, vestibular dysfunction. It is the most common cause of deaf-blindness. Currently cochlear implantation or hearing aids are the only treatments for Usher-related hearing loss. However, gene therapy has shown promise in treating Usher-related retinitis pigmentosa. Here we review how the etiologies of Usher-related hearing loss make it a good candidate for gene therapy and discuss how various forms of gene therapy could be applied to Usher-related hearing loss.     

The effect of increasing application rate of granular calcium ammonium nitrate on net nitrification in a laboratory study of grassland soils

Soil incubation studies were undertaken in controlled environment cabinets at 15°C to investigate the effect of increasing application rates of calcium ammonium nitrate (CAN) on net nitrification in two grassland soils. Granular CAN was applied to the surface of freshly collected, moist soil, at a rate equivalent to 0, 100, 200, 400, 800 and 1600µg NH ₄ ⁺ -N and NO ₃ ^- -N per gram of oven dry soil. In half the treatments finely ground CaCO₃ was incorporated into the moist soil to raise the starting pH. Changes in soil mineral N and pH were measured at weekly intervals up to six-weeks. The most probable number (MPN) technique was used to enumerate the NH ₄ ⁺ -N and NO ₂ ^- -N oxidizers at the beginning and end of the incubation.At low rates of CAN application there was considerable NH ₄ ⁺ -N oxidation to NO ₃ ^- -N during the incubation of both soils. Lime stimulated this N transformation. At high application rates (i.e. 800 and 1600 ppm) there was little change in NH ₄ ⁺ -N or NO ₃ ^- -N on either soil during the 6 week incubation, in the presence or absence of lime. The rate of NO ₃ ^- -N produced peaked at 5.6 and 3.8 mg NO ₃ ^- -N kg^–1 d^–1 on soil 1 and 2 respectively, in the presence of lime. Above a level of 400 ppm CAN (equivalent to 38 kg N ha^–1) the rate of NO ₃ ^- -N produced decreased. The higher rate of net nitrification in soil 1 compared with soil 2 was probably due to a higher number of nitrifying bacteria. Although high rates of CAN decreased the nitrifying activity of both soils there was little difference between treatments in the actual numbers of NH ₄ ⁺ -N and NO ₂ ^- -N oxidizers determined by the MPN technique.The results showed that the rate of granular CAN applied to the soil surface can influence the local activity of nitrifying bacteria and subsequent N transformations. At application rates of CAN generally used agriculturally for grass production, it is likely that net nitrification of the NH ₄ ⁺ -N in the fertilizer granule will be inhibited.     

Abrasion Resistance of Field-Assisted Ion-Exchanged Glass

The fracture strength of HF-etched samples was measured as a function of field-assisted K-Na ion exchange depth, as well as the severity of abrasion. As expected, the more severe the abrasion, the greater the depth required to make the strength insensitive to abrasion. An abrasion-independent weakening mechanism that increased with exchange depth was also found to be operative.     

Diluent effects on carbonate mobility in bisphenol-A polycarbonate in the solid state

The effect of miscible low molecular weight additives on the mobility of the carbonate group in bisphenol-A polycarbonate (BPAPC) has been studied using n.m.r. and dielectric relaxation experiments in the solid state. Proton-enhanced dipolar-decoupled carbon-13 n.m.r. spectra of BPAPC, isotopically enriched at the carbonate position, are obtained without magic-angle sample spinning. The resolved chemical shift anisotropy allows study of nuclear spin relaxation for the carbonate groups in the polymer that have different orientations relative to the static magnetic field in the laboratory frame. The spin-lattice relaxation time in the rotating frame (T_1?) is measured at a motional-probe frequency of 50 kHz for the undiluted polymer and for BPAPC-diluent blends containing either dibutylphthalate or dinitrobiphenyl. The T_1? exhibits some dependence on orientation in all systems studied. In the blend containing dibutylphthalate (DBP), T_1? is decreased by a factor of two for all orientations of the carbonate group. This implies that DBP substantially increases the spectral density of 50 kHz motions in the carbonate region of the polymer at ambient temperature. In contrast, dinitrobiphenyl does not significantly alter the Fourier component of thermal fluctuations at 50 kHz. Dielectric relaxation measurements at 10 kHz reveal that the primary (T_g) and secondary (β) motional processes in BPAPC are affected by low molecular weight additives. An intermediate relaxation process appears in the temperature interval between the glass transition temperature (T_g) and the sub-T_g β-relaxation (T_β) in the polymer-diluent blends. The n.m.r. spin-lattice relaxation rate in the rotating frame, T^?1_1?, correlates well with the relative magnitude of the dielectric dissipation factor (tan δ_ε) between T_g and T_β.     

Advances and future prospects in copper electrowinning

In recent years research on the electrowinning of copper has led to a number of significant advances. These developments include improved mass transfer and higher current density operation through air sparging, reduced anode overvoltage in the conventional cell as a result of cobalt(II) addition to the electrolyte or the use of alternative types of anodes, and the production of high quality cathodes in the electrowinning of copper from solvent extraction strip liquor. The fluidized-bed cathode offers the possibility of continuous electrowinning as well as the direct electrowinning of copper from dilute solutions. The problem of high power consumption may find its solution in the adoption of an alternate anode reaction or in the electrowinning of copper (I) electrolytes. Noteworthy is the development of unique electrowinning cells in conjunction with the hydrometallurgical treatment of copper concentrates. These cells utilize cuprous or ferrous anodic oxidation with the resulting cupric or ferric ions being active lixiviants of sulphide copper minerals. The numerous advances combined with the increasing tonnage of copper being produced by the electrowinning route ensure an interesting and promising future for this process.     

An improved apparatus for oxygen bomb testing

An improved oxygen bomb apparatus has been developed. The increased precision and readability of the continuous pressure recording allows a new method of end-point measurement. It was possible to use smaller test samples than any previously reported. Sample size and oxygen pressure were shown to be relatively unimportant variables. Temperature is the most useful variable for controlling test time. This is preferable over sample dispersion or addition of catalysts in terms of simplifying the procedure. Peroxide formation at the oxygen bomb test end point is three to four times the value at the end point of the Active Oxygen Method. Presented at the AOCS meeting, New Orleans, April 1976. Part I of a series on Evaluation of Antioxidant Performance.     

The effects of bleached and unbleached rosemary oleoresins on light-sensitized oxidation of soybean oil

Bleached and unbleached forms of a rosemary oleoresin (RO) in stripped and nonstripped soybean oil behave both as antioxidant and prooxidant in a light-induced oxidative system. At 0.02 and 0.05% levels, RO had the greatest antioxidant activity, while at 0.01 and 0.5% levels it had the highest prooxidant activity in both stripped and nonstripped soybean oil. Treatment of both soybean oil systems with tertiary butylhydroquinone controlled light-induced oxidation of the oil better than did the oleoresin treatments. The prooxidant activity of the 0.5% RO level was probably due to an excess of prooxidant components being carried into the oil at that level, whereas the reduced antioxidant activity at 0.01% was probably due to the low initial level of active antioxidant components being added to the oil. Published as Journal Series No. 10072, Nebraska Agricultural Research Division, Department of Food Science and Technology, University of Nebraska, Lincoln, NE 68583-0919.     

Structuration bridging diffusion of innovations and gender relations theories: a case of paradigmatic pluralism in IS research

This paper discusses the adoption of a pluralist theoretical framework – one that is also multiparadigmatic – for conducting and publishing information system (IS) research. The discussion is illustrated by a single case study involving the Australian cotton industry. The theoretical framework is informed by three sociological theories, each with its particular paradigmatic assumptions: structuration theory as a meta‐theory, and diffusion of innovations and gender relations as lower‐level theories from notionally opposing paradigms. Theoretical pluralism helped to produce rich findings, illuminating both the social nature of women farmers' roles, the materiality of the cotton farming context, the characteristics of the decision support systems in use and the recursive way in which human agency and institutional pressures shape each other. Because users of so‐called divergent paradigms often face criticism based on the incommensurability issue, one of the main contributions of this paper is to discuss the value of a pluralist and multiparadigmatic theoretical framework in dealing with complex IS social phenomena.     

Relationship between cell morphology and impact strength of microcellular foamed high‐density polyethylene/polypropylene blends

Polymer blends, such as those resulting from recycling postconsumer plastics, often have poor mechanical properties. Microcellular foams have been shown to have the potential to improve properties, and permit higher‐value uses of mixed polymer streams. In this study, the effects of microcellular batch processing conditions (foaming time and temperature) and HDPE/PP blend compositions on the cell morphology (the average cell size and cell‐population density) and impact strength were studied. Optical microscopy was used to investigate the miscibility and crystalline morphology of the HDPE/PP blends. Pure HDPE and PP did not foam well at any processing conditions. Blending facilitated the formation of microcellular structures in polyolefins because of the poorly bonded interfaces of immiscible HDPE/PP blends, which favored cell nucleation. The experimental results indicated that well‐developed microcellular structures are produced in HDPE/PP blends at ratios of 50:50 and 30:70. The cell morphology had a strong relationship with the impact strength of foamed samples. Improvement in impact strength was associated with well‐developed microcellular morphology. Polym. Eng. Sci. 44:1551–1560, 2004. © 2004 Society of Plastics Engineers.     

Aeroelastic tailoring and scaling using Bacterial Foraging Optimisation

Bacterial Foraging Optimisation (BFO) is investigated in an attempt to evaluate its use in solving complex optimisation problems for aeronautical structures. A hybrid variant of BFOA, which incorporates meta-modelling techniques, is also proposed and employed. The efficiency and effectiveness of the methods are tested for tailoring a rectangular composite wing, aiming to maximise the flutter speed and for scaling a joined-wing aircraft, targeting to match aeroelastic responses between the physical prototype and wind tunnel model. The obtained results are compared with those found using a range of other biologically inspired optimisation methods (GA, PSO, ACO), proving that the social foraging behavior of motile bacteria is an effective tool for aeroelastic optimisation.