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  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science Ltd
    Journal of neurochemistry 86 (2003), S. 0 
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Dystonia musculorum (dt) mice suffer from a severe sensory neuropathy caused by mutations in the gene encoding the cytoskeletal cross-linker protein dystonin/bullous pemphigoid antigen 1 (Bpag1). Loss of function of dystonin/Bpag1 within neurons leads to a loss in the maintenance of cytoskeletal organization and to the development of focal axonal swellings prior to death of the neuron. In the present study, we demonstrate that neurons within the sciatic nerves of dt27J mice undergo axonal degeneration as has been previously reported for the dorsal roots. Furthermore, ultrastructural studies reveal a perturbed organization of the neurofilament and microtubule networks within the axons of sciatic nerves in dt27J mice. The disrupted cytoskeletal organization suggested that axonal transport is affected in dt mice. To address this, we assessed fast axonal transport by measuring the rate of accumulation of acetylcholinesterase (AChE) proximal and distal to a surgically introduced ligature on the sciatic nerves of normal and dt27J mice. Our findings demonstrate that axonal transport of AChE in both orthograde and retrograde directions is markedly affected, and allow us to conclude that axonal transport defects do exist in the sciatic nerves of dt27J mice.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Staufen is an RNA-binding protein, first identified for its role in oogenesis and CNS development in Drosophila. Two mammalian homologs of Staufen have been identified and shown to bind double-stranded RNA and tubulin, and to function in the somatodendritic transport of mRNA in neurons. Here, we examined whether Staufen proteins are expressed in skeletal muscle in relation to the neuromuscular junction. Immunofluorescence experiments revealed that Staufen1 (Stau1) and Staufen2 (Stau2) accumulate preferentially within the postsynaptic sarcoplasm of muscle fibers as well as at newly formed ectopic synapses. Western blot analyses showed that the levels of Stau1 and Stau2 are greater in slow muscles than in fast-twitch muscles. Muscle denervation induced a significant increase in the expression of Stau1 and Stau2 in the extrasynaptic compartment of both fast and slow muscles. Consistent with these observations, we also demonstrated that expression of Stau1 and Stau2 is increased during myogenic differentiation and that treatment of myotubes with agrin and neuregulin induces a further increase in the expression of both Staufen proteins. We propose that Stau1 and Stau2 are key components of the postsynaptic apparatus in muscle, and that they contribute to the maturation and plasticity of the neuromuscular junction.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract: The molecular mechanisms underlying the activity-linked plasticity of acetylcholinesterase (AChE) mRNA levels in mammalian skeletal muscle have yet to be established. Here, we demonstrate that denervation of adult muscle induces a dramatic (up to 90%) and rapid (within 24 h) decrease in the abundance of AChE mRNAs. By contrast, denervation of 14-day-old rats leads to a significantly less pronounced reduction (50% of control) in the expression of AChE mRNAs. Assessment of the transcriptional activity of the AChE gene reveals that it remains essentially unchanged in adult denervated muscles, whereas it displays an approximately two- to three-fold increase (p 〈 0.05) in denervated muscles from 2- to 14-day-old rats. In addition, we observed a higher rate of degradation of in vitro transcribed AChE mRNAs upon incubation with protein extracts from denervated muscles. Finally, UV-crosslinking experiments reveal that denervation increases the abundance of RNA-protein interactions in the 3′ untranslated region of AChE transcripts. Taken together, these data suggest that the abundance of AChE transcripts in mature muscles is controlled primarily via posttranscriptional regulatory mechanisms, whereas in neo- and postnatal muscles, both transcriptional and posttranscriptional regulation appears critical in dictating AChE mRNA levels. Accordingly, the activity-linked transcriptional regulation of the AChE gene appears to demonstrate a high level of plasticity during muscle development when maturation of the neuromuscular junctions is still occurring.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Westerville, Ohio : American Ceramics Society
    Journal of the American Ceramic Society 86 (2003), S. 0 
    ISSN: 1551-2916
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics , Physics
    Notes: The structure and composition of sapphire bicrystal boundaries produced by liquid-phase sintering depended on the crystallographic misorientation of the crystals across the boundary and on the orientation of the boundary. Basal twist boundaries of 15° or 30° were not wetted by glass, but contained significant amounts of Ca and Si at the boundary. For tilt boundaries of 7° or 12°, the glass wetted segments of boundaries that contained the basal plane of either crystal. Boundary segments with orientations of 40° or more from the basal plane, however, were dewetted (i.e., “dry”). Boundary segments oriented less than ∼40° from the basal orientation were partially wetted, consisting of segments of wetted and dry grain boundaries. For the 12° tilt boundary, Ca and Si could be detected on portions of the boundary that contained no glass. For bicrystal boundaries having tilts of ≤4°, dewetting occurred for all observed boundary orientations. Basal-oriented segments in these small angle tilt boundaries contained noticeable concentrations of adsorbed Ca and Si, while nonbasal segments were apparently free of Ca and Si. Most results could be explained based on a combined Wulff plot construction, which predicts partially wetted grain boundaries and “missing” angles for unwetted grain boundaries. Results that could not be explained by the construction included growth step ledges bounded by nonequilibrium facet planes.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1551-2916
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics , Physics
    Notes: The effect of grain size on the tensile creep of silicon nitride was investigated on two materials, one containing 5% by volume Yb2O3, the other containing 5% by volume Yb2O3 and 0.5% by mass Al2O3. Annealing of the Al2O3-free silicon nitride for a longer period during processing increased the grain size by a factor of 2. This increase did not affect the tensile creep rate; the grain size exponent of the creep rate differed little from zero, p=−0.20 ± 1.37 (95% confidence level). This finding supports the more recent theories of tensile creep for which p= 0 or −1 and rejects the more classical theory of solution-precipitation. In compression, a more limited data set showed p=−1.89 ± 1.72 (95% confidence level). In contrast to the Al2O3-free material, a longer term anneal of the Al2O3-containing material during processing did not increase its grain size. Despite this, the longer-annealed Al2O3-containing material crept 10 to 100 times slower than the short-annealed material. The enhancement of creep resistance may be a consequence of SiAlON formation during the additional annealing, which reduces the Al content in the amorphous phase and increases its viscosity. Such changes in chemical composition of the grain boundaries are more effective in controlling tensile creep rate than is the grain size.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1551-2916
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics , Physics
    Notes: Cavities formed in ruby (99.46Al2O3·0.54Cr2O3) by the healing and annealing of indentation cracks at 1600°C are more equiaxed than similar cavities in sapphire. Surface energies for the observed facet planes (R, S, and A) relative to the surface energy of the basal plane, C, were γA/C= 1.00 ± 0.03, γR/C= 1.05 ± 0.07, and γS/C= 1.02 ± 0.04, with the uncertainty representing 95% confidence limits. Thus, the surface energies of all observed facets were statistically indistinguishable. Unlike sapphire, P-plane facets were not observed. The substantial rounding of the cavities in ruby indicated that portions of the Wulff shape were above the roughening transition temperature. Thus, even though Cr2O3 and Al2O3 form ideal solutions, Cr3+ ions are sufficiently surface active to modify the relative free energy of the surfaces.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1540-8159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Futura Publishing, Inc.
    Pacing and clinical electrophysiology 25 (2002), S. 0 
    ISSN: 1540-8159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: DARBAR, D., et al.: P Wave Signal-Averaged Electrocardiography to Identify Risk for Atrial Fibrillation. AF is the most common sustained cardiac arrhythmia, and this prevalence is increasing. The impact of AF on morbidity and mortality is substantial, as are the socioeconomic consequences like higher health care costs, chronic disease management, and disabilities. Early recognition of patients at high risk for AF, combined with effective management, may help prevent AF from becoming chronic, helping to minimize potential health risks, costs, and other complications. P wave signal-averaged electrocardiography (SAECG) has been shown to have a potential role in identifying patients at risk of developing paroxysmal AF and those likely to change from paroxysmal AF to chronic AF. The theoretical rationale of P wave SAECG in patients with AF is delay in the intraatrial and interatrial conduction. Intraatrial conduction delays can be seen on the surface electrocardiogram as P wave prolongation, which is more visible with averaging techniques and high resolution recording devices. Averaging followed by amplification after proper filtering of the electrical signal allows more precise measurements of duration and amplitude of the P wave. Data on reproducibility, filter settings, number of beats to be averaged, and definitions of the onset and offset depend on the system used, which can be QRS or, preferably, P wave triggered. A prolonged P wave duration is one of the best predictors of perioperative AF, but the role of P wave SAECG in the paroxysmal form is less well defined. Combining P wave duration with other predictors for AF improves the diagnostic value of P wave SAECG.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    350 Main Street , Malden , MA 02148-5018 , USA and 9600 Garsington Road , Oxford OX4 2DQ , UK . : Blackwell Science Inc
    Pacing and clinical electrophysiology 28 (2005), S. 0 
    ISSN: 1540-8159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Background: Atrial remodeling secondary to atrial fibrillation (AF) may be important in the arrhythmogenic process. Unfortunately, the study of electrophysiologic remodeling in humans has been limited by the invasive nature of most tests of electrophysiologic characteristics. We sought to determine whether changes in atrial electrophysiology occur acutely (within the first hour) after cardioversion and whether these changes could be detected noninvasively by measuring the signal-averaged P-wave. Methods: The filtered P-wave duration (FPD) was measured by signal-averaged electrocardiography (ECG) at 20 and 60 minutes after cardioversion in 46 patients with AF, and the difference between the two values was calculated. The root-mean-square voltage of the terminal 40 ms of the signal-averaged P-wave at 20 and 60 minutes and the difference between them were also determined. Results: The FPD at 20 minutes was significantly different from that at 60 minutes (153.0 ± 19.1 vs 159.7 ± 24.8 ms; P = 0.02). In a univariate linear regression model, none of the clinical variables studied was significantly associated with the change in FPD. The root-mean-square voltage at 20 minutes was not significantly different from that at 60 minutes (5.8 ± 3.0 vs 5.5 ± 2.7; P = 0.14). Conclusions: We conclude that significant changes in atrial electrophysiology occur within the first hour after cardioversion of AF. These changes can be detected by measuring the FPD.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1398-9995
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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