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91.
Schmeisser MJ Ey E Wegener S Bockmann J Stempel AV Kuebler A Janssen AL Udvardi PT Shiban E Spilker C Balschun D Skryabin BV Dieck St Smalla KH Montag D Leblond CS Faure P Torquet N Le Sourd AM Toro R Grabrucker AM Shoichet SA Schmitz D Kreutz MR Bourgeron T Gundelfinger ED Boeckers TM 《Nature》2012,486(7402):256-260
Autism spectrum disorders comprise a range of neurodevelopmental disorders characterized by deficits in social interaction and communication, and by repetitive behaviour. Mutations in synaptic proteins such as neuroligins, neurexins, GKAPs/SAPAPs and ProSAPs/Shanks were identified in patients with autism spectrum disorder, but the causative mechanisms remain largely unknown. ProSAPs/Shanks build large homo- and heteromeric protein complexes at excitatory synapses and organize the complex protein machinery of the postsynaptic density in a laminar fashion. Here we demonstrate that genetic deletion of ProSAP1/Shank2 results in an early, brain-region-specific upregulation of ionotropic glutamate receptors at the synapse and increased levels of ProSAP2/Shank3. Moreover, ProSAP1/Shank2(-/-) mutants exhibit fewer dendritic spines and show reduced basal synaptic transmission, a reduced frequency of miniature excitatory postsynaptic currents and enhanced N-methyl-d-aspartate receptor-mediated excitatory currents at the physiological level. Mutants are extremely hyperactive and display profound autistic-like behavioural alterations including repetitive grooming as well as abnormalities in vocal and social behaviours. By comparing the data on ProSAP1/Shank2(-/-) mutants with ProSAP2/Shank3αβ(-/-) mice, we show that different abnormalities in synaptic glutamate receptor expression can cause alterations in social interactions and communication. Accordingly, we propose that appropriate therapies for autism spectrum disorders are to be carefully matched to the underlying synaptopathic phenotype. 相似文献
92.
Prud'homme B Gompel N Rokas A Kassner VA Williams TM Yeh SD True JR Carroll SB 《Nature》2006,440(7087):1050-1053
The independent evolution of morphological similarities is widespread. For simple traits, such as overall body colour, repeated transitions by means of mutations in the same gene may be common. However, for more complex traits, the possible genetic paths may be more numerous; the molecular mechanisms underlying their independent origins and the extent to which they are constrained to follow certain genetic paths are largely unknown. Here we show that a male wing pigmentation pattern involved in courtship display has been gained and lost multiple times in a Drosophila clade. Each of the cases we have analysed (two gains and two losses) involved regulatory changes at the pleiotropic pigmentation gene yellow. Losses involved the parallel inactivation of the same cis-regulatory element (CRE), with changes at a few nucleotides sufficient to account for the functional divergence of one element between two sibling species. Surprisingly, two independent gains of wing spots resulted from the co-option of distinct ancestral CREs. These results demonstrate how the functional diversification of the modular CREs of pleiotropic genes contributes to evolutionary novelty and the independent evolution of morphological similarities. 相似文献
93.
Global variation in copy number in the human genome 总被引:3,自引:0,他引:3
Redon R Ishikawa S Fitch KR Feuk L Perry GH Andrews TD Fiegler H Shapero MH Carson AR Chen W Cho EK Dallaire S Freeman JL González JR Gratacòs M Huang J Kalaitzopoulos D Komura D MacDonald JR Marshall CR Mei R Montgomery L Nishimura K Okamura K Shen F Somerville MJ Tchinda J Valsesia A Woodwark C Yang F Zhang J Zerjal T Zhang J Armengol L Conrad DF Estivill X Tyler-Smith C Carter NP Aburatani H Lee C Jones KW Scherer SW Hurles ME 《Nature》2006,444(7118):444-454
Copy number variation (CNV) of DNA sequences is functionally significant but has yet to be fully ascertained. We have constructed a first-generation CNV map of the human genome through the study of 270 individuals from four populations with ancestry in Europe, Africa or Asia (the HapMap collection). DNA from these individuals was screened for CNV using two complementary technologies: single-nucleotide polymorphism (SNP) genotyping arrays, and clone-based comparative genomic hybridization. A total of 1,447 copy number variable regions (CNVRs), which can encompass overlapping or adjacent gains or losses, covering 360 megabases (12% of the genome) were identified in these populations. These CNVRs contained hundreds of genes, disease loci, functional elements and segmental duplications. Notably, the CNVRs encompassed more nucleotide content per genome than SNPs, underscoring the importance of CNV in genetic diversity and evolution. The data obtained delineate linkage disequilibrium patterns for many CNVs, and reveal marked variation in copy number among populations. We also demonstrate the utility of this resource for genetic disease studies. 相似文献
94.
Haislip JB Nysewander MC Reichart DE Levan A Tanvir N Cenko SB Fox DB Price PA Castro-Tirado AJ Gorosabel J Evans CR Figueredo E MacLeod CL Kirschbrown JR Jelinek M Guziy S de Ugarte Postigo A Cypriano ES LaCluyze A Graham J Priddey R Chapman R Rhoads J Fruchter AS Lamb DQ Kouveliotou C Wijers RA Bayliss MB Schmidt BP Soderberg AM Kulkarni SR Harrison FA Moon DS Gal-Yam A Kasliwal MM Hudec R Vitek S Kubanek P Crain JA Foster AC Clemens JC Bartelme JW Canterna R Hartmann DH Henden AA Klose S 《Nature》2006,440(7081):181-183
Gamma-ray bursts (GRBs) and their afterglows are the most brilliant transient events in the Universe. Both the bursts themselves and their afterglows have been predicted to be visible out to redshifts of z approximately 20, and therefore to be powerful probes of the early Universe. The burst GRB 000131, at z = 4.50, was hitherto the most distant such event identified. Here we report the discovery of the bright near-infrared afterglow of GRB 050904 (ref. 4). From our measurements of the near-infrared afterglow, and our failure to detect the optical afterglow, we determine the photometric redshift of the burst to be z = 6.39 - 0.12 + 0.11 (refs 5-7). Subsequently, it was measured spectroscopically to be z = 6.29 +/- 0.01, in agreement with our photometric estimate. These results demonstrate that GRBs can be used to trace the star formation, metallicity, and reionization histories of the early Universe. 相似文献
95.
Induction of an illusory shadow person 总被引:1,自引:0,他引:1
Stimulation of a site on the brain's left hemisphere prompts the creepy feeling that somebody is close by. The strange sensation that somebody is nearby when no one is actually present has been described by psychiatric and neurological patients, as well as by healthy subjects, but it is not understood how the illusion is triggered by the brain. Here we describe the repeated induction of this sensation in a patient who was undergoing presurgical evaluation for epilepsy treatment, as a result of focal electrical stimulation of the left temporoparietal junction: the illusory person closely 'shadowed' changes in the patient's body position and posture. These perceptions may have been due to a disturbance in the multisensory processing of body and self at the temporoparietal junction. 相似文献
96.
Pedro R. Bartholomay Kevin A. Williams Roberto A. Cambra Marcio L. Oliveira 《Journal of Natural History》2019,53(43-44):2639-2683
ABSTRACTTraumatomutilla duplicata duplicata (Gerstaecker, 1874), T. duplicata feia Casal, 1969, T. bruchi André, 1908, T. cristata (Gerstaecker, 1874), T. aterrima (Gerstaecker, 1874) and T. lorena (Cresson, 1902) are proposed as junior synonyms of Traumatomutilla bivittata (Gerstaecker, 1874). Traumatomutilla immaculiceps André, 1901, T. bivittata rubroguttata André, 1901 and T. estrella (Cresson, 1902) are proposed as junior synonyms of T. juvenilis (Gerstaecker, 1874). Traumatomutilla bispiculata (André, 1907) is proposed as a junior synonym of T. miniata (Gerstaecker, 1874). The previously unknown males of T. guarata Casal, 1969 and T. juvenilis are described and illustrated. All species of the T. juvenilis species group are redescribed and illustrated. A new species, Traumatomutilla juvenindica Bartholomay & Williams sp. nov., is described based on males and females from northern South America. Additionally, identification keys to the species and known colour forms of the T. juvenilis species group are provided.http://www.zoobank.org/urn:lsid:zoobank.org:pub:3563B9EE-A45B-4939-8436-7A808D4F8996 相似文献
97.
ABSTRACTTwo species of bopyrid isopods of the Bopyrinae subfamily are recorded from new localities and hosts in northeastern Brazil. Parabopyrella lata (Nierstrasz and Brender à Brandis, 1929) was recorded from the state of Ceará, found for the first time parasitising the caridean shrimp Alpheus packardii Kingsley, 1880. In addition, Probopyrus cf. pandalicola (Packard, 1879) is recorded from the state of Bahia, parasitising the palaemonid shrimp Palaemon northropi (Rankin, 1898). Taxonomic comments are provided for each species. 相似文献
98.
Lambert Williams 《Studies in history and philosophy of science》2005,36(1):23-41
Cardano’s Liber de ludo aleae is subjected to a reappraisal based largely on considerations of practice in the 16th century gambling arena. It is argued that Cardano’s purported failure to secure the foundations of a rigorous probability calculus can be explained as something that occurred precisely because of his gambling exposure, not in spite of it. 相似文献
99.
本刊2005年第5期发表的冯长根教授“学术期刊编辑应该承担起科技创新的历史责任”一文,在国内外引起了反响。远在美国的一位物理学家法瑞斯·E·威廉斯先生专门发来电子邮件对此问题进行讨论,并发表了自己的意见。本刊特将此信翻译发表,以期引起更多读者关注并参与讨论,促使我国科技期刊在科技创新中发挥更大的作用。 相似文献
100.
Pang SS Berry R Chen Z Kjer-Nielsen L Perugini MA King GF Wang C Chew SH La Gruta NL Williams NK Beddoe T Tiganis T Cowieson NP Godfrey DI Purcell AW Wilce MC McCluskey J Rossjohn J 《Nature》2010,467(7317):844-848
The pre-T-cell antigen receptor (pre-TCR), expressed by immature thymocytes, has a pivotal role in early T-cell development, including TCR β-selection, survival and proliferation of CD4(-)CD8(-) double-negative thymocytes, and subsequent αβ T-cell lineage differentiation. Whereas αβTCR ligation by the peptide-loaded major histocompatibility complex initiates T-cell signalling, pre-TCR-induced signalling occurs by means of a ligand-independent dimerization event. The pre-TCR comprises an invariant α-chain (pre-Tα) that pairs with any TCR β-chain (TCRβ) following successful TCR β-gene rearrangement. Here we provide the basis of pre-Tα-TCRβ assembly and pre-TCR dimerization. The pre-Tα chain comprised a single immunoglobulin-like domain that is structurally distinct from the constant (C) domain of the TCR α-chain; nevertheless, the mode of association between pre-Tα and TCRβ mirrored that mediated by the Cα-Cβ domains of the αβTCR. The pre-TCR had a propensity to dimerize in solution, and the molecular envelope of the pre-TCR dimer correlated well with the observed head-to-tail pre-TCR dimer. This mode of pre-TCR dimerization enabled the pre-Tα domain to interact with the variable (V) β domain through residues that are highly conserved across the Vβ and joining (J) β gene families, thus mimicking the interactions at the core of the αβTCR's Vα-Vβ interface. Disruption of this pre-Tα-Vβ dimer interface abrogated pre-TCR dimerization in solution and impaired pre-TCR expression on the cell surface. Accordingly, we provide a mechanism of pre-TCR self-association that allows the pre-Tα chain to simultaneously 'sample' the correct folding of both the V and C domains of any TCR β-chain, regardless of its ultimate specificity, which represents a critical checkpoint in T-cell development. This unusual dual-chaperone-like sensing function of pre-Tα represents a unique mechanism in nature whereby developmental quality control regulates the expression and signalling of an integral membrane receptor complex. 相似文献