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Mice that are homozygous with respect to a mutation (ax(J)) in the ataxia (ax) gene develop severe tremors by 2-3 weeks of age followed by hindlimb paralysis and death by 6-10 weeks of age. Here we show that ax encodes ubiquitin-specific protease 14 (Usp14). Ubiquitin proteases are a large family of cysteine proteases that specifically cleave ubiquitin conjugates. Although Usp14 can cleave a ubiquitin-tagged protein in vitro, it is unable to process polyubiquitin, which is believed to be associated with the protein aggregates seen in Parkinson disease, spinocerebellar ataxia type 1 (SCA1; ref. 4) and gracile axonal dystrophy (GAD). The physiological substrate of Usp14 may therefore contain a mono-ubiquitin side chain, the removal of which would regulate processes such as protein localization and protein activity. Expression of Usp14 is significantly altered in ax(J)/ax(J) mice as a result of the insertion of an intracisternal-A particle (IAP) into intron 5 of Usp14. In contrast to other neurodegenerative disorders such as Parkinson disease and SCA1 in humans and GAD in mice, neither ubiquitin-positive protein aggregates nor neuronal cell loss is detectable in the central nervous system (CNS) of ax(J) mice. Instead, ax(J) mice have defects in synaptic transmission in both the central and peripheral nervous systems. These results suggest that ubiquitin proteases are important in regulating synaptic activity in mammals.  相似文献   
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The immune system consists of two evolutionarily different but closely related responses, innate immunity and adaptive immunity. Each of these responses has characteristic receptors-Toll-like receptors (TLRs) for innate immunity and antigen-specific receptors for adaptive immunity. Here we show that the caspase recruitment domain (CARD)-containing serine/threonine kinase Rip2 (also known as RICK, CARDIAK, CCK and Ripk2) transduces signals from receptors of both immune responses. Rip2 was recruited to TLR2 signalling complexes after ligand stimulation. Moreover, cytokine production in Rip2-deficient cells was reduced on stimulation of TLRs with lipopolysaccharide, peptidoglycan and double-stranded RNA, but not with bacterial DNA, indicating that Rip2 is downstream of TLR2/3/4 but not TLR9. Rip2-deficient cells were also hyporesponsive to signalling through interleukin (IL)-1 and IL-18 receptors, and deficient for signalling through Nod proteins-molecules also implicated in the innate immune response. Furthermore, Rip2-deficient T cells showed severely reduced NF-kappaB activation, IL-2 production and proliferation on T-cell-receptor (TCR) engagement, and impaired differentiation to T-helper subtype 1 (TH1) cells, indicating that Rip2 is required for optimal TCR signalling and T-cell differentiation. Rip2 is therefore a signal transducer and integrator of signals for both the innate and adaptive immune systems.  相似文献   
346.
Marine aerosol formation from biogenic iodine emissions   总被引:6,自引:0,他引:6  
The formation of marine aerosols and cloud condensation nuclei--from which marine clouds originate--depends ultimately on the availability of new, nanometre-scale particles in the marine boundary layer. Because marine aerosols and clouds scatter incoming radiation and contribute a cooling effect to the Earth's radiation budget, new particle production is important in climate regulation. It has been suggested that sulphuric acid derived from the oxidation of dimethyl sulphide is responsible for the production of marine aerosols and cloud condensation nuclei. It was accordingly proposed that algae producing dimethyl sulphide play a role in climate regulation, but this has been difficult to prove and, consequently, the processes controlling marine particle formation remains largely undetermined. Here, using smog chamber experiments under coastal atmospheric conditions, we demonstrate that new particles can form from condensable iodine-containing vapours, which are the photolysis products of biogenic iodocarbons emitted from marine algae. Moreover, we illustrate, using aerosol formation models, that concentrations of condensable iodine-containing vapours over the open ocean are sufficient to influence marine particle formation. We suggest therefore that marine iodocarbon emissions have a potentially significant effect on global radiative forcing.  相似文献   
347.
In this work we present a novel approach for describing radio signal spaces for localization algorithms.We first introduce a new metric, the Discretely Distributed Log-H ¨older Metric(DDLHM). The DDLHM is designed to characterize the type and degree of signal distortion relative to lognormal signal-to-distance path models. We first show how the DDLHM can describe and discriminate distortions in an exhaustive set of synthetic signal spaces.We then determine a reduced set of maximally diagnostic distortion parameters. Using only 4% of the maximal set of DDLHMs, we found the reduced set matches with an acceptable degree of error 95% of the time. Using the synthetic reduced set, we characterized a variety of wireless localization algorithms' behaviors to attenuation, bias,and multipath. We found algorithms made much different tradeoffs between best case and average case error. We then use the DDLHM to identify distortion types in three different physical environments using measured 802.11 signal strengths, and predict the positioning performance of several localization algorithms. Our approach predicts average localization error to within 2 meters of the observed average error.  相似文献   
348.
The present study analyzed the stomach contents of 65 Great Basin spadefoot toads, Spea intermontana (Cope), collected in an area of irrigation runoff in south central Washington State. Toads were collected by pitfall trapping. Traps were maintained for 1 year beginning in April 2002. Toads consumed at least 56 different arthropod taxa belonging to the orders Coleoptera, Diptera, Hemiptera, Hymenoptera, Lepidoptera, Neuroptera, Orthoptera, Trichoptera, Collembola, and Araneae. Ants and darkling beetles were among the most common prey. Feeding appeared to be very generalized with the toads accepting almost anything they could capture and subdue.  相似文献   
349.
The life cycle of Cheumatopsyche analis (Banks) is described from 2 urban streams located in north central Colorado. Larvae were collected every 15 days during spring, summer, and fall and every 30 days in winter from August 1999 through August 2000. A total of 8652 larvae were collected, and head capsules were measured to the nearest 0.01 mm. The life cycle of C. analis at both sites appears to be partially bivoltine with a long, almost continuous period of recruitment after peak emergence in June. Some offspring from the early generation that emerges in May probably emerge as adults in September, while the remainder of the population overwinter as instars III through V. Life cycle patterns for C. analis in these 2 urban streams were most similar to other populations of Cheumatopsyche from Virginia.  相似文献   
350.
This study explores how the relationship between flow and riparian vegetation varies along a montane river. We mapped occurrence of woody riparian plant communities along 58 km of the San Miguel River in southwestern Colorado. We determined the recurrence interval of inundation for each plant community by combining step-backwater hydraulic modeling at 4 representative reaches with Log-Pearson analysis of 4 stream gaging stations. Finally, we mapped bottomland surficial geology and used a Geographic Information System to overlay the coverages of geology and vegetation. Plant communities were distinctly arrayed along the hydrologic gradient. The Salix exigua Nuttall (sandbar willow) community occurred mostly on surfaces with a recurrence interval of inundation shorter than 2.2 years; the Betula occidentalis Hooker (river birch) community peaked on sites with recurrence intervals of inundation between 2.2 and 4.6 years. The hydrologic position occupied by communities dominated by Populus angustifolia James (narrowleaf cottonwood) was strongly related to age of trees and species composition of understory shrubs. The fraction of riparian vegetation on surfaces historically inundated by the river decreased in the upstream direction from almost 100% near Uravan to <50% along the South Fork of the San Miguel River. In upstream reaches much of the physical disturbance necessary to maintain riparian vegetation is provided by valley-side processes including debris flows, floods from minor tributaries, landslides, and beaver activity. Where valley-side processes are important, prediction of riparian vegetation change based on alterations of river flow will be incomplete.  相似文献   
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