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11.
Leukoencephalopathy with vanishing white matter (VWM) is an inherited brain disease that occurs mainly in children. The course is chronic-progressive with additional episodes of rapid deterioration following febrile infection or minor head trauma. We have identified mutations in EIF2B5 and EIF2B2, encoding the epsilon- and beta-subunits of the translation initiation factor eIF2B and located on chromosomes 3q27 and 14q24, respectively, as causing VWM. We found 16 different mutations in EIF2B5 in 29 patients from 23 families. We also found two distantly related individuals who were homozygous with respect to a missense mutation in EIF2B2, affecting a conserved amino acid. Three other patients also had mutations in EIF2B2. As eIF2B has an essential role in the regulation of translation under different conditions, including stress, this may explain the rapid deterioration of people with VWM under stress. Mutant translation initiation factors have not previously been implicated in disease.  相似文献   
12.
Climate change and population declines in a long-distance migratory bird   总被引:1,自引:0,他引:1  
Both C  Bouwhuis S  Lessells CM  Visser ME 《Nature》2006,441(7089):81-83
Phenological responses to climate change differ across trophic levels, which may lead to birds failing to breed at the time of maximal food abundance. Here we investigate the population consequences of such mistiming in the migratory pied flycatcher, Ficedula hypoleuca. In a comparison of nine Dutch populations, we find that populations have declined by about 90% over the past two decades in areas where the food for provisioning nestlings peaks early in the season and the birds are currently mistimed. In areas with a late food peak, early-breeding birds still breed at the right time, and there is, at most, a weak population decline. If food phenology advances further, we also predict population declines in areas with a late food peak, as in these areas adjustment to an advanced food peak is insufficient. Mistiming as a result of climate change is probably a widespread phenomenon, and here we provide evidence that it can lead to population declines.  相似文献   
13.
The Nuffield Council on Bioethics suggests that introgression of genetic material into related species in centres of crop biodiversity is an insufficient justification to bar the use of genetically modified crops in the developing world. They consider that a precautionary approach to forgo the possible benefits invokes the fallacy of thinking that doing nothing is itself without risk to the poor. Here we report findings relevant to this and other aspects of environmental biosafety for genetically modified potato in its main centre of biodiversity, the central Andes. We studied genetically modified potato clones that provide resistance to nematodes, principal pests of Andean potato crops. We show that there is no harm to many non-target organisms, but gene flow occurs to wild relatives growing near potato crops. If stable introgression were to result, the fitness of these wild species could be altered. We therefore transformed the male sterile cultivar Revolucion to provide a genetically modified nematode-resistant potato to evaluate the benefits that this provides until the possibility of stable introgression to wild relatives is determined. Thus, scientific progress is possible without compromise to the precautionary principle.  相似文献   
14.
机械活化粉末制备W-Cu合金的微观组织   总被引:5,自引:2,他引:5  
为了改善制备工艺和提高W-Cu合金的密度与性能,对原料粉末进行了机械活化处理,通过成形和烧结制备了W-Cu合金,考察了活化后粉末的变化,观察了合金的烧结组织并测量了密度.结果表明,机械活化能增大钨铜粉末的表面能和晶格畸变能,有效地促进烧结,改善烧结组织;烧结体中存在大量团絮状组织,团粒内部钨颗粒主要以固相方式烧结在一起,而团粒之间则以液相烧结为主.烧结组织细密、均匀,相对密度达98%以上;烧结组织中钨铜两相在微米尺度下仍然结合良好,部分界面出现了互溶.  相似文献   
15.
Theoretical studies have shown that variation in density regulation strongly influences population dynamics, yet our understanding of factors influencing the strength of density dependence in natural populations still is limited. Consequently, few general hypotheses have been advanced to explain the large differences between species in the magnitude of population fluctuations. One reason for this is that the detection of density regulation in population time series is complicated by time lags induced by the life history of species that make it difficult to separate the relative contributions of intrinsic and extrinsic factors to the population dynamics. Here we use population time series for 23 bird species to estimate parameters of a stochastic density-dependent age-structured model. We show that both the strength of total density dependence in the life history and the magnitude of environmental stochasticity, including transient fluctuations in age structure, increase with generation time. These results indicate that the relationships between demographic and life-history traits in birds translate into distinct population dynamical patterns that are apparent only on a scale of generations.  相似文献   
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断陷盆地构造枢纽部位油气富集规律   总被引:1,自引:0,他引:1  
以十屋断陷为例,根据其构造演化、主要断层特征及油气成藏条件分析,揭示多旋回断陷盆地构造枢纽带与油气富集关系.结果表明:十屋断陷枢纽部位经历了断陷期、断拗过渡期、拗陷期、萎缩隆升期等复杂的构造演化,多期差异构造变形及断裂活动导致了小宽枢纽带南段和北段具有相似性及差异性,后期构造反转发育部位多为早期断层发育部位,且主要集中在主干断层上,构造运动产生位移被断裂和褶皱吸收;不同阶段的隆起部位相叠加,使枢纽部位与构造高点相对位置发生变化,枢纽部位相对位置由原来的古隆起演变为斜坡部位;枢纽部位早期为油气的有利指向区,晚期具有良好的保存条件,同时发育多类型圈闭、有利沉积相组合及生储盖组合,围绕枢纽带可形成复式油气聚集带.  相似文献   
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19.
Summary The skin of the African frog, Hylambates maculatus, contains large amounts of a caerulein-like peptide, which has been identified as Asn2, Leu5-caerulein. The cholecystokinetic activity of Hylambates-caerulein is very similar to that of caerulein.Supported in part by grants from the Consiglio Nazionale delle Ricerche, Roma, Italy.  相似文献   
20.
L M Dalderup  W Visser 《Nature》1969,222(5198):1050-1052
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