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Sreedharan J Blair IP Tripathi VB Hu X Vance C Rogelj B Ackerley S Durnall JC Williams KL Buratti E Baralle F de Belleroche J Mitchell JD Leigh PN Al-Chalabi A Miller CC Nicholson G Shaw CE 《Science (New York, N.Y.)》2008,319(5870):1668-1672
Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disorder characterized pathologically by ubiquitinated TAR DNA binding protein (TDP-43) inclusions. The function of TDP-43 in the nervous system is uncertain, and a mechanistic role in neurodegeneration remains speculative. We identified neighboring mutations in a highly conserved region of TARDBP in sporadic and familial ALS cases. TARDBPM337V segregated with disease within one kindred and a genome-wide scan confirmed that linkage was restricted to chromosome 1p36, which contains the TARDBP locus. Mutant forms of TDP-43 fragmented in vitro more readily than wild type and, in vivo, caused neural apoptosis and developmental delay in the chick embryo. Our evidence suggests a pathophysiological link between TDP-43 and ALS. 相似文献
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Zusammenfassung Es wurden die Wirkungen von Precocen I (PI) unter Laborverhältnissen auf die frühen L4 und Puppen vonCulex quinquefasciatus bei Konzentrationen von 0,001–10,0 ppm untersucht. Das Spektrum der PI-Wirkungen schloß die Larven-und Adulten-Mortalität bei 0,1–10,0 ppm ein. Die Verpuppungs-und Adultenschlüpf-Periode wurden bei Dosen von 0,001–5,0 ppm verzögert. Auf die larvalen Nachkommen vonC. qu. wirkte sich die PI-Behandlung (0,001–10,0 ppm) in verringerter Fekundität und Eischlüpfquote, verzögerte Larvenentwicklung und schließlichem Absterben aus.
Mit einer Abbildung und 4 Tabellen 相似文献
Effects of precocene I onCulex quinquefasciatus Say (Diptera, Culicidae)
The effects of precocene-I (PI) onCulex quinquefasciatus Say were studied under laboratory conditions by treating laboratory reared early 4th instar larvae and pupae with 0.001–10.0 ppm of PI. The range of PI effects included larval and adult mortalities at 0.1–10.0 ppm. Pupation and adult emergence periods were delayed at 0.001–5.0 ppm. Effects of PI were maintained to offsprings ofC. quinquefasciatus. These effects involved reduced fecundity, reduced egg hatchability and delayed larval offspring development and finally the death at 0.001–10.0 ppm of PI.
Mit einer Abbildung und 4 Tabellen 相似文献
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Distribution of Vehicular Lead in Roadside Soils of Major Roads of Brisbane,Australia 总被引：1，自引：0，他引：1
Collections were made from three sites in the urbanarea of Brisbane, Australia. At each site, soilsamples were collected from transects parallel andperpendicular to the roadway, as well as a depthprofile. Total lead was determined by refluxing thesamples with concentrated nitric acid (1 h) andorganic lead by shaking with cold ammoniacal methanol(Flameless AAS). Both chloride and bromide anionswere obtained from an aqueous extract (HPLC). Resultsshowed that vehicular emissions were the major sourceof lead in the roadside soils of this study. At siteslocated in relatively enclosed areas of higheratmospheric stability, both lead and bromide contentsdecreased markedly with increasing distance from theroadway. However, in the absence of such atmosphericstability, the distribution of both lead and bromidecontents revealed different patterns which wasattributed mainly to the effect of meteorologicalfactors such as wind direction and speed. The depthprofile of roadside soils generally showed leadaccumulation within the uppermost 5 cm, whereaselevated concentrations of bromide were observed at greater depth. 相似文献
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