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71.
72.
Smith-Lemli-Opitz syndrome (SLOS), desmosterolosis and lathosterolosis are human syndromes caused by defects in the final stages of cholesterol biosynthesis. Many of the developmental malformations in these syndromes occur in tissues and structures whose embryonic patterning depends on signaling by the Hedgehog (Hh) family of secreted proteins. Here we report that response to the Hh signal is compromised in mutant cells from mouse models of SLOS and lathosterolosis and in normal cells pharmacologically depleted of sterols. We show that decreasing levels of cellular sterols correlate with diminishing responsiveness to the Hh signal. This diminished response occurs at sterol levels sufficient for normal autoprocessing of Hh protein, which requires cholesterol as cofactor and covalent adduct. We further find that sterol depletion affects the activity of Smoothened (Smo), an essential component of the Hh signal transduction apparatus.  相似文献   
73.
Neuropathy target esterase (NTE) is involved in neural development and is the target for neurodegeneration induced by selected organophosphorus pesticides and chemical warfare agents. We generated mice with disruptions in Nte, the gene encoding NTE. Nte(-/-) mice die after embryonic day 8, and Nte(+/-) mice have lower activity of Nte in the brain and higher mortality when exposed to the Nte-inhibiting compound ethyl octylphosphonofluoridate (EOPF) than do wild-type mice. Nte(+/-) and wild-type mice treated with 1 mg per kg of body weight of EOPF have elevated motor activity, showing that even minor reduction of Nte activity leads to hyperactivity. These studies show that genetic or chemical reduction of Nte activity results in a neurological phenotype of hyperactivity in mammals and indicate that EOPF toxicity occurs directly through inhibition of Nte without the requirement for Nte gain of function or aging.  相似文献   
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75.
Macroinvertebrate communities were examined on 4 winter dates over a 4-yr period in 2 high-altitude Rocky Mountain streams to document overwintering assemblages potentially experiencing spring acid pulses. Taxa richness values were comparable to other published lists for alpine and montane stream systems despite the fact that most literature reflected summer collections. Mean benthic density ranged from 1406 to 19,734 organisms/m 2 , and drift rates ranged from 0 to 1740 organisms/100 m 3 . Benthic collections showed higher taxa richness than drift collections while the Ephemeroptera and Plecoptera occurred in greater proportions in drift than in benthos. The Nemouridae (Plecoptera), Ephemerellidae and Heptageniidae (Ephemeroptera), Chironomidae (Diptera), and Hydracarina were the numerically dominant taxa in benthic collections. Grazer/scrapers and shredder/detritivores were always the numerically dominant functional feeding groups at all sites, composing 60-90% of the benthos. Predators, constituting approximately 15% of the community, occurred in the same relative proportion at all sites on all dates. Winter macroinvertebrate communities in these low-order, montane streams exhibit high taxonomic richness and benthic densities as great as lower-elevation mountain streams in the West.  相似文献   
76.
Rabbit abundance patterns in the North American Southwest are poorly known, particularly relative to the potentially important influences of rainfall and plant production. We conducted a 10-year study on the comparative densities of black-tailed jackrabbits ( Lepus californicus ) and desert cottontail rabbits ( Sylvilagus audubonii ) in relation to annual rainfall and plant production in both black grama ( Bouteloua eriopoda ) grassland and creosotebush ( Larrea tridentata ) shrubland habitats in the northern Chihuahuan Desert. We found that both species of rabbits were more abundant in black grama grassland than in creosotebush shrubland and that plant production was positively correlated with rainfall at both habitats. However, rabbit densities were not positively correlated with rainfall or plant production in either habitat. In fact, both species of rabbits exhibited gradual, multiyear changes in abundance rather than the patterns of high annual variation observed in desert rodent communities. Our findings of no significant relationship between plant production and rabbit densities indicate that rabbit abundance in the northern Chihuahuan Desert may not be strongly regulated by short-term, bottom-up effects of annual variation in plant production. Long-term trends indicate that other factors such as disease or predation may be equally or more important. We suggest that future research evaluate both top-down and bottom-up influences to elucidate the temporal dynamics of Chihuahuan Desert rabbit abundance. Se conoce poco sobre los patrones de abundancia de conejos desérticos en el suroeste de Norte America, particularmente en relación con las influencias potencialmente importantes de la precipitación y la producción de plantas. Llevamos a cabo una investigación de diez años sobre la densidad relativa de la liebre de California ( Lepus californicus ) y el conejo cola de algodón ( Sylvilagus audubonii ) en los hábitats tanto de pradera de grama negra ( Bouteloua eriopoda ) como de matorral de gobernadora ( Larrea tridentata ) en el norte del Desierto Chihuahuense, en relación con la precipitación y la producción anuales. Encontramos que ambas especies de conejo fueron más abundantes en la pradera de grama negra que en el matorral de gobernadora, y que la producción de plantas estuvo correlacionada positivamente con la precipitación en ambos hábitats. Sin embargo, las densidades de conejos no estuvieron correlacionadas positivamente con la precipitación o con la producción de plantas en ninguno de los dos hábitats. De hecho, en cuanto a la fluctuación de su abundancia, las dos especies de conejo exhibieron tendencias graduales a lo largo de varios años, en comparación con los patrones de gran variación anual que se han observado en comunidades de roedores desérticos. La falta de una relación significativa entre la producción de plantas y la abundancia de conejos, indica que la abundancia de conejos en el norte del Desierto Chihuahuense no está fuertemente regulada por los efectos ascendentes a corto plazo de la variación anual en la producción de plantas. Los cambios reflejados en las tendencias a largo plazo indican que otros factores como las enfermedades o la depredación pueden ser de igual o mayor importancia, y sugerimos que investigaciones futuras deberán evaluar tanto las influencias ascendentes como las descendentes para entender las dinámicas temporales en la abundancia de conejos del Desierto Chihuahuense.  相似文献   
77.
设计了一个非专业人士能够使用、最简化的气候敏感模型,用来研究人类活动所导致的全球变暖的幅度问题.在1990年的第一次IPCC评估报告中,IPCC对其报告中预测的未来全球变暖幅度很有信心,但是随后的观测结果显示全球的变暖幅度只有预测的一半.而自2001年起,全球变暖出现停滞,但是仅仅考虑到二氧化碳浓度的增加,很少有模型能够模拟出这一变化.在已出版的IPCC第五次评估报告的草稿中,IPCC大幅度削减了近期变暖的幅度,并以专家评估代替了模型预测.但是报告中关于未来气候长期变化的预测仍被保留.如果把IPCC模型的总反馈从1.9 W m–2 K–1调整到1.5 W m–2 K–1,气候敏感模型中模拟的温度将从3.2 K降至2.2 K.同时由于反馈很可能是净负反馈,更合适的估计应该是1.0 K.1.0 K是一个能够实现的增幅,21世纪的实际变暖将会小于1 K.即使燃烧所有可开采的化石燃料也不会使全球变暖的幅度超过2.2 K,这一增加幅度也将趋于平稳.本文认为解决IPCC第四、五次报告中评估方法的差异非常关键.一旦这些差异得到解决,人类活动导致的全球变暖在22世纪以及几个世纪以后的平稳态将有可能不会超过IPCC当前模型预测的1/3~1/2.  相似文献   
78.
There has been a resurgence in applying bioassessment techniques for evaluating and monitoring the biological integrity of stream ecosystms. In all cases biological metrics have been refined to account for regional variation in aquatic habitats and fauna. This study evaluated environmental and macroinvertebrate properties for wadable streams in 3 major ecoregions of Idaho: Northern Basin and Range, Snake River Plain, and Northern Rocky Mountain. These 3 ecoregions constitute > 80% of the land area in Idaho. Reference streams were delineated from test streams in each ecoregion using standard habitat assessment protocols. (Plafkin et al. 1989). Multiple discriminant analysis effectively determined habitat (quantified measures) and macroinvertebrate differences between reference and test streams within ecoregions, although the results suggested that quantifiable habitat measures (e.g., water chemistry and nutrients) and biotic measures based on taxonomic groups (e.g., % Elmidae) improved the discriminatory power of evaluation procedures. Our results support the contention of a multi-metric approach for assessing differences among streams within an ecoregion. Lastly, individual metrics differed in their importance for evaluating stream condition among ecoregions, further emphasizing the importance of regionally stratifying metric selection or scoring procedures.  相似文献   
79.
We compared functional attributes of streams draining catchments burned by wildfire 20 years previously to those of streams in unburned catchments. Long-term analyses of channel profiles indicated most channel change occurred within the first 10 years after fire with little subsequent change the following 10 years. Much of the standing dead timber had fallen, and its effect on stream morphology was directly related to stream size, with important ramifications for future years as decay progresses. The volume of wood in the active channel was 5X higher in a 3rdorder burn stream than in other burn or reference streams, but > 80% of this wood was still bridging the stream. Retention of leaves was strongly associated with channel morphology and location of debris dams. Sediment respiration was significantly greater (1.7X) in streams of burned catchments, resulting from greater amounts of loosely attached organic matter in the sediments of these streams. In concordance with respiration results, coefficients of exchange (k ex ) were almost 5X higher in burn streams than in reference streams, although estimates of transient storage were similar between stream types. We expect the input of large woody debris to increase in the next 10 years in fire-impacted streams as bridging trees collapse into the stream, thereby enhancing channel complexity and habitat heterogeneity, instream metabolism and retention, and consequently stream function. The results emphasize the importance of landscape history, such as large-scale wildfires, on present patterns and processes in stream ecosystems.  相似文献   
80.
We summarized historic and recent fish distributions in South Dakota and analyzed fish faunal similarity at 2 spatial scales (geomorphic province and river drainage) for both historic (native) and recent (post-1990) faunas. We quantified zoogeographic patterns between geomorphic provinces and among neighboring river drainages for historic and recent faunas. We also quantified faunal change (species losses and additions) between provinces and among drainages. Ninety-seven fishes were native to South Dakota, but 111 fishes were present in recent collections because 8 native species were missing, and 22 nonnatives were present. There was high β diversity among historic and recent river drainage fish faunas, but there was between 22% and 56% faunal change between periods. Recent faunas were homogenized compared to historic faunas at both provincial and river drainage spatial scales. Patterns of nonnative species establishment were geographically distinct from patterns of native species loss. Most nonnative species additions were in cold-water or human-made habitats of the Great Plains. Most native species declines stemmed from warm water streams of the Central Lowlands and Missouri River valley. Conservation of rare and declining native species and containment of nonnatives are both necessary to preserve historical patterns of fish biodiversity in South Dakota.  相似文献   
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