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Mutations in a P-type AtPase gene cause axonal degeneration

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Mutations in a P-type ATPase gene cause axonal degeneration

Fine Mapping of a Deafness Mutation hml on Mouse Chromosome 10

2012全国发育生物学大会

作者： Richard t.Libby Wilhelmine N.de Vries Richard S.Smith Dana L.Wright roderick t.bronson Kevin L.Seburn Simon W.M.John the Howard Hughes Medical Institute The Jackson Laboratory600 Main StreetBar HarborMEUSA Department of Ophthalmology Tufts University of MedicineBostonMAUSA

Neuronal loss and axonal degeneration are important pathological features of many neurodegenerative diseases. the molecular mechanisms underlying the majority of axonal degeneration conditions remain *** better understand axonal degeneration,we studied a mouse mutant wabbler-Iethal(wl).Wabbler-lethal(wl) mutant mice develop progressive ataxia with pronounced neurodegeneration in the central and peripheral nervous *** studies have led to a debate of whether myelinopathy or axonopathy is the primary cause of neurodegeneration observed in wl mice. Here we provide clear evidence that wabbler lethal mutants develop an axonopathy,and that this axonopathy is modulated by Wld and Bax *** addition,we have identified the gene harboring the disease-causing mutations as *** studied three wl alleles and found that all result from mutations in the Atp8a2 *** analysis shows that AtP8A2 possesses phosphatidylserine translocase activity and is involved in localization of phosphatidylserine to the inner leaflet of the plasma ***8a2 is widely expressed in the brain,the spinal cord,and *** assessed two of the mutant alleles of Atp8a2 and found they are both nonfunctional for the phosphatidylserine translocase activity. Conclusions:Our data demonstrate for the first time that mutation of a mammalian phosphatidylserine translocase causes axon degeneration and neurodegenerative disease.

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Journal of Pharmaceutical Analysis 2004年第1期16卷 91-91页

作者： Belinda S Harris Patricia F Ward-Bailey roderick t bronson Muriel t Davisson Kenneth R Johnson the Jackson Laboratory The Jackson LaboratoryThe Jackson LaboratoryThe Jackson LaboratoryThe Jackson Laboratory Bar Harbor Maine 04609 USA Bar Harbor Maine 04609 USA Bar Harbor Maine 04609 USA Bar Harbor Maine 04609 USA Bar Harbor Maine 04609 USA

Objective to map a mouse deafness gene, identify the underlying mutation and develop a mouse model for human deafness. Methods Genetic linkage cross and genome scan were used to map a novel mutation named hypoplasia of the membranous labyrinth (hml), which causes hearing loss in mutant mice. Results ① hml was mapped on mouse Chr 10 (～43 cM from the centromere) suggests that the homologous human gene is on 12q22-q24, which was defined on the basis of known mouse-human homologies (OMIM, 2004). ② this study has generated 25 polymorphic microsatellite markers, placed 3 known human genes in the correct order in a high-resolution mouse map and narrowed the hml candidate gene region to a 500 kb area.

关键词： mouse deafness mutation

A review of direct numerical simulations of astrophysical detonations and their implications

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Frontiers of physics 2013年第2期8卷 189-198页

作者： Suzanne t. Parete-Koon Christopher R. Smith thomas L. Papatheodore O. E. bronson Messer National Center for Computational Sciences Oak Ridge National Laboratory Oak Ridge USA TN 37831-6354 Department of Physics and Astronomy University of tennessee KnoxvilleUSA TN 37996-1200

Multi-dimensional direct numerical simulations （DNS） of astrophysical detonations in degenerate matter have revealed that the nuclear burning is typically characterized by cellular structure caused by transverse instabilities in the detonation front. type Ia supernova modelers often use one- dimensional DNS of detonations as inputs or constraints for their whole star simulations. While these one-dimensional studies are useful tools, the true nature of the detonation is multi-dimensional. the multi-dimensional structure of the burning influences the speed, stability, and the composition of the detonation and its burning products, and therefore, could have an impact on the spectra of type Ia supernovae. Considerable effort has been expended modeling type Ia supernovae at densities above 1 ~ 107 ***-3 where the complexities of turbulent burning dominate the flame propagation. How- ever, most full star models turn the nuclear burning schemes off when the density falls below 1 ~ 107 ***-3 and distributed burning begins. the deflagration to detonation transition （DDt） is believed to occur at just these densities and consequently they are the densities important for studying the properties of the subsequent detonation. this work will review the status of DNS studies of detona- tions and their possible implications for type Ia supernova models. It will cover the development of Detonation theory from the first simple Chapman-Jouguet （C J） detonation models to the current models based on the time-dependent, compressible, reactive flow Euler equations of fluid dynamics.

关键词： supernova detonations direct numerical simulations

Global Asymptotics of Orthogonal Polynomials Associated with a Generalized Freud Weight

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Chinese Annals of Mathematics,Series B 2018年第3期39卷 553-596页

作者： Zhi-tao WEN roderick WONG Shuai-Xia XU Department of Mathematics Taiyuan University of TechnologyTaiyuan 030024China Liu Bie Ju Centre for Mathematical Sciences City University of Hong KongHong KongChina Institut Franco-Chinois de l’Energie Nucleaire Sun Yat-sen UniversityGuangzhou 510275China

In this paper, the authors consider the asymptotic behavior of the monic polynomials orthogonal with respect to the weight function w（x） = /x/2αe-（x4＋tx2）, x ∈R, where α is a constant larger than - 1/2 and t is any real number. they consider this problem in three separate cases：（i） c 〉 -2, （ii） c = -2, and （iii） c 〈 -2, where c ：= tN-1/2 is a constant, N = n ＋ a and n is the degree of the polynomial. In the first two cases, the support of the associated equilibrium measure μ is a single interval, whereas in the third case the support of μt consists of two intervals. In each case, globally uniform asymptotic expansions are obtained in several regions. these regions together cover the whole complex plane. the approach is based on a modified version of the steepest descent method for Riemann-Hilbert problems introduced by Deift and Zhou （1993）.

关键词： Orthogonal polynomials Globally uniform asymptotics Riemann-Hilbertproblems the second Painlev6 transcendent theta function

Spatially and spectrally resolved orbital angular momentum interactions in plasmonic vortex generators

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Light(Science & Applications) 2019年第1期8卷 907-915页

作者： Jordan A.Hachtel Sang-Yeon Cho roderick B.Davidson II Matthew A.Feldman Matthew F.Chisholm Richard F.Haglund Juan Carlos Idrobo Sokrates t.Pantelides Benjamin J.Lawrie Center for Nanophase Materials Science Oak Ridge National LaboratoryOak RidgeTN 37831USA Klipsch School of Electrical and Computer Engineering New Mexico State UniversityLas CrucesNM 88003USA Department of Physics and Astronomy Vanderbilt UniversityNashvilleTN 37235USA Quantum Information Science Group Oak Ridge National LaboratoryOak RidgeTN 37831USA Materials Science and technology Division Oak Ridge National LaboratoryOak RidgeTN 37831USA Department of Electrical Engineering and Computer Science Vanderbilt University NashvilleNashvilleTN 37235USA Chemistry Division U.S.Naval Research LaboratoryWashingtonD.C.20375USA

Understanding the near-field electromagnetic interactions that produce optical orbital angular momentum(OAM)is crucial for integrating twisted light into ***,we examine the cathodoluminescence(CL)of plasmonic vortices carrying OAM generated in spiral *** nanospiral geometry defines a photonic local density of states that is sampled by the electron probe in a scanning transmission electron microscope(StEM),thus accessing the optical response of the plasmonic vortex with high spatial and spectral *** map the full spectral dispersion of the plasmonic vortex in spiral structures designed to yield increasing topological ***,we fabricate nested nanospirals and demonstrate that OAM from one nanospiral can be coupled to the nested nanospiral,resulting in enhanced luminescence in concentric spirals of like handedness with respect to concentric spirals of opposite *** results illustrate the potential for generating and coupling plasmonic vortices in chiral nanostructures for sensitive detection and manipulation of optical OAM.

关键词： momentum spectral angular

the Prognostic Value of Myocardial Deformation in Patients with Congenital Aortic Stenosis

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Congenital Heart Disease 2021年第3期16卷 221-232页

作者： roderick W.J.van Grootel Allard t.van den Hoven Dan Bowen tijmen Ris Jolien W.Roos-Hesselink Annemien E.van den Bosch Department of Cardiology Erasmus Medical CentreRotterdamThe Netherlands

Aims:to assess the prognostic value of left ventricular(LV)global longitudinal strain(GLS)and global longitudinal early diastolic strain rate(GLSre)with regard to cardiovascular events,as congenital aortic stenosis(AoS)is associated with significant mortality and morbidity but predictors for clinical outcome are *** analysis provides a robust and reproducible method for early detection of LV dysfunction,which might be of prognostic ***:this prospective study,included clinically stable patients with congenital AoS between 2011–*** GLS and GLSre was performed in the apical 4,3 and 2-chamber views using tomtec *** endpoint was a composite of death,heart failure,hospitalization,arrhythmia,thrombo-embolic events and ***:In total 138 patients were included(33[26–43]years,86(62%)male),NYHA class I:134(97%).Mean LV GLS was–15.3±3.2%,GLSre 0.66±0.18 s–*** correlated with Nt-proBNP,LV volumes and ejection fraction(strongest LV GLS with LV EF:r–0.539,ptrongest LV GLSre with age:r–0.376,pthe endpoint occurred in 53(38%)patients:4 patients died,9 developed heart failure,22 arrhythmias,8 thrombo-embolic events and 35 *** LV GLS(standardized HR(sHR 0.62(95%CI 0.47–0.81)and GLSre(sHR 0.62(95%CI 0.47–0.83)were associated with the *** multivariable analysis showed that both GLS and GLSre were associated independent of left atrial volume,Nt-proBNP and prior ***:Left ventricular GLS and GLSre are reduced in adult patients with congenital *** markers are associated with adverse cardiac events and have clear clinical relevance.

关键词： Speckle tracking echocardiography congenital aortic stenosis strain prognosis

Longitudinal variations of electron temperature and total ion density in the sunset equatorial topside ionosphere

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Longitudinal variations of electron temperature and total io...

Systolic and Diastolic Strain Measurements Show Left Ventricular Dysfunction in Women with turner Syndrome

中国科学院地质与地球物理研究所2008学术年会

作者： roderick A.Heelis Hanson Center for Space Sciences University of Texas at DallasRichardsonTexasUSA.

[1]Based on the DMSP F13 Satellite observations from 1995 to 2005,the longitudinal distributions of the electron temperature(t) and total ion density(N) in the sunset equatorial topside ionosphere are examined. the results suggest that the longitudinal variations of both t and N exhibit obvious seasonal dependence as follows: (1)wavenumber-four longitudinal structure in equinox, (2)three peaks structure in June solstice,and(3) two peaks structure in December ***,the longitudinal variations of t and N show significant anti-correlation,and we speculate that the longitudinal variation of t may result from that of N which can control t through the electron cooling *** wavenumber-four longitudinal structures of both t and N in equinox may relate to the eastward propagating zonal wavenumber-3 diurnal tide(DE3),which has effect on the amplitude of the daytime zonal electric field. the longitudinal variation of t and N in the two solstices may be caused both by longitudinal variation of geomagnetic declination and DE3.

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Congenital Heart Disease 2021年第4期16卷 357-368页

作者： Allard t.van den Hoven Sultan Yilmazer Lidia R.Bons roderick W.Jvan Grootel L.W.Geenen An M.van Berendoncks Anthonie L.Duijnhouwer Hans-Marc J.Siebelink Jolien W.Roos-Hesselink Annemien E.van den Bosch Department of Congenital Cardiology Erasmus MCRotterdamNetherlands Department of Cardiology Radboud University Medical CenterNijmegenNetherlands Department of Cardiology Leiden University Medical CenterRotterdamNetherlands

Aim:this study aimed to describe the systolic left ventricular global longitudinal strain(LvGLS)and left ventricular peak early diastolic strain rate(Sre)in adult women with turner syndrome(tS)and to determine its relationship with exercise capacity and clinical ***:In this cross-sectional cohort study,consecutively included adult tS women underwent an electrocardiogram,transthoracic echocardiogram(ttE)and cardiopulmonary exercise test(CPEt)on the same *** and Sre were measured using 2D speckle tracking analysis(StE)and compared with age-matched healthy female ***:Ninety-four adult women(age 36±13 years)with tS and 32 healthy age-matched female controls were *** with tS had a significantly impaired/reduced systolic LvGLS(–17.82±2.98%vs.–21.80±1.85%,p0.001),compared to healthy female ***,tS women had reduced diastolic function as measured by conventional echocardiographic parameters:A higher A-wave(ptio(p=0.001),longer deceleration time(p=0.006),and a higher E/E’-ratio(pth tS also had a significantly reduced maximal workload(p=0.033),reduced oxygen uptake(pt rate(pthat Age,karyotype and Qt-duration were significantly associated with Sre,but not with LvGLS,in the tS ***:Systolic and diastolic strain and exercise capacity were significantly reduced in tS women compared to healthy *** correlation between strain itself and exercise capacity could be demonstrated,but correlations with conventional ttE parameters and baseline characteristics were found.

关键词： turnersyndrome speckletracking echocardiography strain exercise capacity

StUDY ON LEGAL ISSUES OF tHE URBAN AND RURAL CONStRUCtION LAND REPLACEMENt:FROM tHE PERSPECtIVE OF NEW-tYPE RIGHtS

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China Legal Science 2019年第5期7卷 52-90页

作者： Sun Jianwe Robert D. roderick(Revised) Koguan School of Law of Shanghai Jiao tong University Law School of Anhui University of Finance and Economics 不详

Faced with the needs of farmland protection and urbanization, many areas have carried out urban and rural construction land replacement under the existing land system, which gives birth to land exploitation rights and land development rights. Land exploitation rights belong to landowners and they are sources of changes of land use. Based on that, land development rights determine the attribution of relevant appreciation. Appreciation of homestead where indicators are delivered belongs to homestead users while appreciation of other construction land is shared by local government, collective and all members. In areas where indicators are received, collective members have priority in homestead exploitation rights. the local government can enjoy the appreciation. Appreciation from other rural construction land should be shared by the collective, collective members and local government. Asset gains from collective resources belong to the collective. After replacement, homestead users ' rights on land remain constant or turn into rights to use urban construction land which can renew automatically and freely. Non-collective members can only be assignees of superficies whose term is shorter than construction lands for urban housing.

关键词：城乡建设用地土地权利法律问题置换宅基地使用权土地发展权土地开发权建设用地使用权