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281.
Although 70 per cent of global crustal magmatism occurs at mid-ocean ridges-where the heat budget controls crustal structure, hydrothermal activity and a vibrant biosphere-the tempo of magmatic inputs in these regions remains poorly understood. Such timescales can be assessed, however, with natural radioactive-decay-chain nuclides, because chemical disruption to secular equilibrium systems initiates parent-daughter disequilibria, which re-equilibrate by the shorter half-life in a pair. Here we use 210Pb-226Ra-230Th radioactive disequilibria and other geochemical attributes in oceanic basalts less than 20 years old to infer that melts of the Earth's mantle can be transported, accumulated and erupted in a few decades. This implies that magmatic conditions can fluctuate rapidly at ridge volcanoes. 210Pb deficits of up to 15 per cent relative to 226Ra occur in normal mid-ocean ridge basalts, with the largest deficits in the most magnesium-rich lavas. The 22-year half-life of 210Pb requires very recent fractionation of these two uranium-series nuclides. Relationships between 210Pb-deficits, (226Ra/230Th) activity ratios and compatible trace-element ratios preclude crustal-magma differentiation or daughter-isotope degassing as the main causes for the signal. A mantle-melting model can simulate observed disequilibria but preservation requires a subsequent mechanism to transport melt rapidly. The likelihood of magmatic disequilibria occurring before melt enters shallow crustal magma bodies also limits differentiation and heat replenishment timescales to decades at the localities studied. 相似文献
282.
A critical requirement for diverse applications in quantum information science is the capability to disseminate quantum resources over complex quantum networks. For example, the coherent distribution of entangled quantum states together with quantum memory (for storing the states) can enable scalable architectures for quantum computation, communication and metrology. Here we report observations of entanglement between two atomic ensembles located in distinct, spatially separated set-ups. Quantum interference in the detection of a photon emitted by one of the samples projects the otherwise independent ensembles into an entangled state with one joint excitation stored remotely in 10(5) atoms at each site. After a programmable delay, we confirm entanglement by mapping the state of the atoms to optical fields and measuring mutual coherences and photon statistics for these fields. We thereby determine a quantitative lower bound for the entanglement of the joint state of the ensembles. Our observations represent significant progress in the ability to distribute and store entangled quantum states. 相似文献
283.
284.
MYCN haploinsufficiency is associated with reduced brain size and intestinal atresias in Feingold syndrome 总被引:3,自引:0,他引:3
van Bokhoven H Celli J van Reeuwijk J Rinne T Glaudemans B van Beusekom E Rieu P Newbury-Ecob RA Chiang C Brunner HG 《Nature genetics》2005,37(5):465-467
Feingold syndrome is characterized by variable combinations of esophageal and duodenal atresias, microcephaly, learning disability, syndactyly and cardiac defect. We show here that heterozygous mutations in the gene MYCN are present in Feingold syndrome. All mutations are predicted to disrupt both the full-length protein and a new shortened MYCN isoform, suggesting that multiple aspects of early embryogenesis and postnatal brain growth in humans are tightly regulated by MYCN dosage. 相似文献
285.
Vega H Waisfisz Q Gordillo M Sakai N Yanagihara I Yamada M van Gosliga D Kayserili H Xu C Ozono K Jabs EW Inui K Joenje H 《Nature genetics》2005,37(5):468-470
Roberts syndrome is an autosomal recessive disorder characterized by craniofacial anomalies, tetraphocomelia and loss of cohesion at heterochromatic regions of centromeres and the Y chromosome. We identified mutations in a new human gene, ESCO2, associated with Roberts syndrome in 15 kindreds. The ESCO2 protein product is a member of a conserved protein family that is required for the establishment of sister chromatid cohesion during S phase and has putative acetyltransferase activity. 相似文献
286.
Sample damage by X-rays and other radiation limits the resolution of structural studies on non-repetitive and non-reproducible structures such as individual biomolecules or cells. Cooling can slow sample deterioration, but cannot eliminate damage-induced sample movement during the time needed for conventional measurements. Analyses of the dynamics of damage formation suggest that the conventional damage barrier (about 200 X-ray photons per A2 with X-rays of 12 keV energy or 1 A wavelength) may be extended at very high dose rates and very short exposure times. Here we have used computer simulations to investigate the structural information that can be recovered from the scattering of intense femtosecond X-ray pulses by single protein molecules and small assemblies. Estimations of radiation damage as a function of photon energy, pulse length, integrated pulse intensity and sample size show that experiments using very high X-ray dose rates and ultrashort exposures may provide useful structural information before radiation damage destroys the sample. We predict that such ultrashort, high-intensity X-ray pulses from free-electron lasers that are currently under development, in combination with container-free sample handling methods based on spraying techniques, will provide a new approach to structural determinations with X-rays. 相似文献
287.
288.
Identification of the familial cylindromatosis tumour-suppressor gene 总被引:25,自引:0,他引:25
Bignell GR Warren W Seal S Takahashi M Rapley E Barfoot R Green H Brown C Biggs PJ Lakhani SR Jones C Hansen J Blair E Hofmann B Siebert R Turner G Evans DG Schrander-Stumpel C Beemer FA van Den Ouweland A Halley D Delpech B Cleveland MG Leigh I Leisti J Rasmussen S 《Nature genetics》2000,25(2):160-165
Familial cylindromatosis is an autosomal dominant genetic predisposition to multiple tumours of the skin appendages. The susceptibility gene (CYLD) has previously been localized to chromosome 16q and has the genetic attributes of a tumour-suppressor gene (recessive oncogene). Here we have identified CYLD by detecting germline mutations in 21 cylindromatosis families and somatic mutations in 1 sporadic and 5 familial cylindromas. All mutations predict truncation or absence of the encoded protein. CYLD encodes three cytoskeletal-associated-protein-glycine-conserved (CAP-GLY) domains, which are found in proteins that coordinate the attachment of organelles to microtubules. CYLD also has sequence homology to the catalytic domain of ubiquitin carboxy-terminal hydrolases (UCH). 相似文献
289.
van Bokhoven H Celli J Kayserili H van Beusekom E Balci S Brussel W Skovby F Kerr B Percin EF Akarsu N Brunner HG 《Nature genetics》2000,25(4):423-426
Robinow syndrome is a short-limbed dwarfism characterized by abnormal morphogenesis of the face and external genitalia, and vertebral segmentation. The recessive form of Robinow syndrome (RRS; OMIM 268310), particularly frequent in Turkey, has a high incidence of abnormalities of the vertebral column such as hemivertebrae and rib fusions, which is not seen in the dominant form. Some patients have cardiac malformations or facial clefting. We have mapped a gene for RRS to 9q21-q23 in 11 families. Haplotype sharing was observed between three families from Turkey, which localized the gene to a 4. 9-cM interval. The gene ROR2, which encodes an orphan membrane-bound tyrosine kinase, maps to this region. Heterozygous (presumed gain of function) mutations in ROR2 were previously shown to cause dominant brachydactyly type B (BDB; ref. 7). In contrast, Ror2-/- mice have a short-limbed phenotype that is more reminiscent of the mesomelic shortening observed in RRS. We detected several homozygous ROR2 mutations in our cohort of RRS patients that are located upstream from those previously found in BDB. The ROR2 mutations present in RRS result in premature stop codons and predict nonfunctional proteins. 相似文献
290.
Mutations in ARHGEF6, encoding a guanine nucleotide exchange factor for Rho GTPases, in patients with X-linked mental retardation 总被引:18,自引:0,他引:18
Kutsche K Yntema H Brandt A Jantke I Nothwang HG Orth U Boavida MG David D Chelly J Fryns JP Moraine C Ropers HH Hamel BC van Bokhoven H Gal A 《Nature genetics》2000,26(2):247-250
X-linked forms of mental retardation (XLMR) include a variety of different disorders and may account for up to 25% of all inherited cases of mental retardation. So far, seven X-chromosomal genes mutated in nonspecific mental retardation (MRX) have been identified: FMR2, GDI1, RPS6KA3, IL1RAPL, TM4SF2, OPHN1 and PAK3 (refs 2-9). The products of the latter two have been implicated in regulation of neural plasticity by controlling the activity of small GTPases of the Rho family. Here we report the identification of a new MRX gene, ARHGEF6 (also known as alphaPIX or Cool-2), encoding a protein with homology to guanine nucleotide exchange factors for Rho GTPases (Rho GEF). Molecular analysis of a reciprocal X/21 translocation in a male with mental retardation showed that this gene in Xq26 was disrupted by the rearrangement. Mutation screening of 119 patients with nonspecific mental retardation revealed a mutation in the first intron of ARHGEF6 (IVS1-11T-->C) in all affected males in a large Dutch family. The mutation resulted in preferential skipping of exon 2, predicting a protein lacking 28 amino acids. ARHGEF6 is the eighth MRX gene identified so far and the third such gene to encode a protein that interacts with Rho GTPases. 相似文献