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101.
Laurance D. Smith Catherine E. Kenny Steven P. Ringel Hans E. Neville 《The Western journal of medicine》1988,148(4):430-432
We saw 166 patients with motor neuron disease over a ten-year period, 116 with amyotrophic lateral sclerosis–111 sporadic and 5 familial–and 50 with progressive muscular atrophy. The age at onset varied widely, with the youngest mean onset occurring in the familial group. The most common presenting symptoms were leg or arm weakness and difficulty speaking or swallowing; fewer patients reported cramping, fasciculation, or fatigue. Mean survival time was less in familial cases, women, older patients, and in those with difficulty speaking and swallowing. A total of 50% of all patients were alive after four years; 13% were alive after ten years. Previous reports on the natural history of motor neuron disease may be overly pessimistic in suggesting that survival time rarely exceeds two years. 相似文献
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Vicky Chaar Sandrine Laurance Claudine Lapoumeroulie Sylvie Cochet Maria De Grandis Yves Colin Jacques Elion Caroline Le Van Kim Wassim El Nemer 《The Journal of biological chemistry》2014,289(16):11512-11521
Vaso-occlusive crises are the main acute complication in sickle cell disease. They are initiated by abnormal adhesion of circulating blood cells to vascular endothelium of the microcirculation. Several interactions involving an intricate network of adhesion molecules have been described between sickle red blood cells and the endothelial vascular wall. We have shown previously that young sickle reticulocytes adhere to resting endothelial cells through the interaction of α4β1 integrin with endothelial Lutheran/basal cell adhesion molecule (Lu/BCAM). In the present work, we investigated the functional impact of endothelial exposure to hydroxycarbamide (HC) on this interaction using transformed human bone marrow endothelial cells and primary human pulmonary microvascular endothelial cells. Adhesion of sickle reticulocytes to HC-treated endothelial cells was decreased despite the HC-derived increase of Lu/BCAM expression. This was associated with decreased phosphorylation of Lu/BCAM and up-regulation of the cAMP-specific phosphodiesterase 4A expression. Our study reveals a novel mechanism for HC in endothelial cells where it could modulate the function of membrane proteins through the regulation of phosphodiesterase expression and cAMP-dependent signaling pathways. 相似文献
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JULIEN GRANGIER ALAIN DEJEAN PIERRE-JEAN G. MALÉ PASCAL-JEAN SOLANO JÉRÔME ORIVEL 《Biological journal of the Linnean Society. Linnean Society of London》2009,97(1):90-97
In the understory of pristine Guianese forests, the myrmecophyte Hirtella physophora almost exclusively shelters colonies of the plant-ant Allomerus decemarticulatus in its leaf pouches. We experimentally tested three non-mutually exclusive hypotheses concerning phenomena that can determine the species specificity of this association throughout the foundation stage of the colonies: (1) interspecific competition results in the overwhelming presence of A. decemarticulatus queens or incipient colonies; (2) exclusion filters prevent other ant species from entering the leaf pouches; and (3) host-recognition influences the choice of founding queens, especially A. decemarticulatus . Neither interspecific competition, nor the purported exclusion filters that we examined play a major role in maintaining the specificity of this association. Unexpectedly, the plant trichomes lining the domatia appear to serve as construction material during claustral foundation rather than as a filter. Finally, A. decemarticulatus queens are able to identify their host plant from a distance through chemical and/or visual cues, which is rarely demonstrated in studies on obligatory ant–plant associations. We discuss the possibility that this specific host-recognition ability could participate in shaping a compartmentalized plant-ant community where direct competition between ant symbionts is limited. © 2009 The Linnean Society of London, Biological Journal of the Linnean Society , 2009, 97 , 90–97. 相似文献
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The Iberian Peninsula represents a hot spot of cyphophthalmid (mite harvestman) disparity, with four of the eight genera currently recognized in the family Sironidae represented in the region – a generic diversity and morphological disparity not found in any other region of the World so far. From these, two genera (Iberosiro and Odontosiro) are monotypic, and are restricted to the western side of the peninsula. Parasiro is restricted to the north‐east region, from the Catalonian Coastal Ranges and both sides of the Eastern Pyrenees, in areas where the annual rainfall surpasses 1000 mm, and mostly restricted to areas with Paleozoic and Variscan rocks, with other species of the genus extending to Corsica, Sardinia, and the Italian Peninsula. A second species of the genus Paramiopsalis, Paramiopsalis eduardoi sp. nov. from Fragas do Eume, is described here along with a re‐diagnosis of the genus. Paramiopsalis species, together with Odontosiro, inhabit the north‐west corner of the Iberian Peninsula, an area with some of the highest recorded annual rainfall, and with Paleozoic rocks from the Iberian Massif or Variscan granitoid rocks. A phylogenetic analysis of the members of the family Sironidae using four molecular markers, despite not including all of the Iberian genera, clearly shows the non‐monophyly of the Iberian Cyphophthalmi, indicating that the Iberian Peninsula is home to multiple ancient lineages of mite harvestmen. The two Paramiopsalis species form a sister clade to the Balkan genus Cyphophthalmus, whereas Parasiro constitutes the first lineage of the sironids represented. 相似文献
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We assessed a general hypothesis of tropical tree diversity that predicts that species richness will be positively correlated with stand dynamism. Our analysis was based one of the largest and longest-running datasets on Amazonian trees (≥10 cm diameter at breast height), with data collected over a 23-yr period within 66 1 ha plots spanning a large (1000 km2) landscape. Within these plots, maximum tree-species richness (329 species/ha) and Fisher's α values (227.5) were among the highest ever recorded. Contrary to the diversity-dynamism concept, tree species richness in our landscape was significantly and negatively associated with stand dynamism (measured as the mean rate of annual tree turnover). Because of this unexpected finding, we critically re-evaluated the relationship between stand dynamism and tree diversity across the Amazon basin and the tropics as a whole. With the inclusion of additional data we found that the relationship between stand dynamism and tree diversity becomes non-significant at larger spatial scales. 相似文献