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71.
Risheg H Graham JM Clark RD Rogers RC Opitz JM Moeschler JB Peiffer AP May M Joseph SM Jones JR Stevenson RE Schwartz CE Friez MJ 《Nature genetics》2007,39(4):451-453
Opitz-Kaveggia syndrome (also known as FG syndrome) is an X-linked disorder characterized by mental retardation, relative macrocephaly, hypotonia and constipation. We report here that the original family for whom the condition is named and five other families have a recurrent mutation (2881C>T, leading to R961W) in MED12 (also called TRAP230 or HOPA), a gene located at Xq13 that functions as a thyroid receptor-associated protein in the Mediator complex. 相似文献
72.
Gout AM;ADPKD Gene Variant Consortium Ravine D Harris PC Rossetti S Peters D Breuning M Henske EP Koizumi A Inoue S Shimizu Y Thongnoppakhun W Yenchitsomanus PT Deltas C Sandford R Torra R Turco AE Jeffery S Fontes M Somlo S Furu LM Smulders YM Mercier B Ferec C Burtey S Pei Y Kalaydjieva L Bogdanova N McCluskey M Geon LJ Wouters CH Reiterova J Stekrová J San Millan JL Aguiari G Del Senno L 《Nature genetics》2007,39(4):427-428
73.
A QTL for rice grain width and weight encodes a previously unknown RING-type E3 ubiquitin ligase 总被引:59,自引:0,他引:59
Grain weight is one of the most important components of grain yield and is controlled by quantitative trait loci (QTLs) derived from natural variations in crops. However, the molecular roles of QTLs in the regulation of grain weight have not been fully elucidated. Here, we report the cloning and characterization of GW2, a new QTL that controls rice grain width and weight. Our data show that GW2 encodes a previously unknown RING-type protein with E3 ubiquitin ligase activity, which is known to function in the degradation by the ubiquitin-proteasome pathway. Loss of GW2 function increased cell numbers, resulting in a larger (wider) spikelet hull, and it accelerated the grain milk filling rate, resulting in enhanced grain width, weight and yield. Our results suggest that GW2 negatively regulates cell division by targeting its substrate(s) to proteasomes for regulated proteolysis. The functional characterization of GW2 provides insight into the mechanism of seed development and is a potential tool for improving grain yield in crops. 相似文献
74.
1.INTaODUCTIONThemultilayerfeedforwardneuralnetworks(MFNN)hasbeenwidelyappliedinmanyfieldssuchaspatternrecognition,nonlinearoptimization,systemidentificationandcontrol.Thesimpleback-propagation(BP)learningalgorithmisusuallyadoptedtoupdateitsweights.TheBPalgorithm,however,suffersdrawbacksofslowconvergenceandunpredictablesolutionsduringlearning.Althoughmailyapproachessuchasvariablestepsize,conjugategradient,pseudo-Newtonmethods[4]andothermethods[3,10],havebeendevelopedtoovercometheproble… 相似文献
75.
Jian CHEN Yunhui LIU Xiping SONG School of Economics ManagementTsinghua University Beijing P.R. China Department of System Engineering Engineering ManagementThe Chinese University of Hong Kong Shatin NT Hong Kong P.R China 《系统科学与系统工程学报(英文版)》2004,13(2):202-218
In this paper we consider a group-buying online auction (GBA) model for a monopolistic manufacturer selling novel products in the uncertain market. Firstly, we introduce the bidder's dominant strategy, after which we optimize the GBA price curve and the production volume together. Finally, we compare the GBA with the traditional posted pricing mechanism and find that the GBA is highly probable to be advantageous over the posted pricing mechanism in some appropriate market environments. 相似文献
76.
77.
Zhongsheng WANG Zhigang ZENG Xiaoxin LIAODepartment of Control Science Engineering Huazhong University of Science Technology Wuhan Hubei P. R. China 《系统科学与系统工程学报(英文版)》2003,12(2):204-209
Some stability criteria are obtained for a class of uncertain systems with time-delay usingLyapunov functional and analytic techniques. It is easy to check the criteria by making use of theboundedness of the uncertainties. 相似文献
78.
1.INTRODUCTIONThealgorithmforconstrainedoptimizationincomplexsystemscanbedividedintotwoclasses:exactandheuristicmethods.Theexactmethods,whicharepartlysummarizedbyTitanetal.[1],havecomputationalrequiremeatsthatgrowexponentiajlywiththesizeoftheproblem.AlthoughheuristicmethodshavemodestcomputationalrequirementsandareeasytoAnplemellt,theyaregenerallyforredundancyallocation[2-4].2.S~EMENTOFProBLEM2.1NotationN:nUmberofcomponents;M:numberofconstraints;Rs:systemreliability;Rj:thereliabili… 相似文献
79.
1.INTRODUCTION2-statesystemreliabilityoftenassumesthatthesystemisrepresentedbyaprobabilisticgraphG=(V,E),withasetV~{yi,v2,'',}ofvenicesandasetE~{el,e2,'',}ofedges(orlinks,branches)containedinit.Thesystemisfunctioningifthereexistsapathfromtheinputnodetotheoutputnode.Thusthereliabilityisconsideredwithamatterofconnectivityonlyandthereliabilityanalysishasbeenprimarilyconcernedwiththeenumerationofpathesorcutsinthereferences[1-4].Butinmanyphysicalsystemsuchaspowertransmissionsystems,oilorwat… 相似文献
80.
Rong Ximin 《系统工程与电子技术(英文版)》1999,(4)
1.INTRODUCTIONInsurancepricingisaneternalthemeinthestudyofinsurance.Whenwedesignainsuranceproduct,wemusttogiveapricefortheproductinordertoselltheproductininsurancemarkets.Whatiscalledinsurancepricingisactuallytomakeinsurancepremiumofinsuranceproduct.Reasonablepricingofinsuranceproductisofutmostimportancefortheexistenceanddevelopmentofinsurancecompany.Ifpremiumismadetoohigh-priced,althoughinsurancecompanycanobtainhighprofitofunderweightingadcanheightencapacityofinsurancecompanyinpaymentofd… 相似文献