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Gene therapy.for spinal muscular atrophy.perspectives on the possibility.of optimizing SMN1 delivery.to correct all neurological and sy.temic perturbations

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Neural Regeneration Research 2025年第7期20卷 2011-2012页

作者： Sharon j.Brown Rafael j.y.ñez-Muñoz Heidi R.Fuller School of Pharmacy.and Bioengineering Keele UniversityKeeleUK Wolfson Center for Inherited Neuromuscular Disease TORCH BuildingRJAH Orthopaedic HospitalOswestryUK *** Center of Gene and Cell TherapyDepartment of Biological SciencesSchool of Life Sciences and the EnvironmentRoyal Holloway University of LondonEghamUK

Spinal muscular atrophy.SMA)is a genetic condition that results in selective lower motor neuron loss with concomitant muscle weakness and *** genetic cause of SMA was understood in 1995 when loss or impairment of the survival motor neuron 1(SMN1)gene was identified as the main contributing factor(Lefebvre et al.,1995).This,in combination with the discovery.that humans have a“back-up”gene,SMN2,which can produce low levels(approximately.10%)of the full-length functional SMN protein,has led to the generation of SMA-specific gene *** was traditionally.classified according to age of sy.ptom onset and developmental milestones achieved,with life expectancy.and severity.vary.ng between ***,SMN2 copy.number is used as a proxy.for the prediction of disease severity.with higher SMN2 copy.number ty.ically.being associated with reduced severity.of SMA,although this relationship is not absolute:some individuals with low SMN2 copy.number have less severe SMA phenoty.es and vice ***,the etiology.of SMA is further complicated by.other factors,such as non-ty.ical nucleotide variants and SMN2-independent modifiers of disease severity.

关键词： atrophy traditionally absolute

On the Bloch Constant for K-Quasiconformal Mappings in Several Complex Variables

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Acta Mathematica Sinica,English Series 2001年第2期17卷 237-242页

作者： j. y. GAMALIEL Department of Mathematics. Nanj.ng Normal University. Nanj.ng 210097. P. R. China

We study.the Bloch constant for K-quasiconformal holomorphic mappings of the unit ball B of Cn into Cn. The final result we prove in this paper is: If f is a K-quasiconformal holomorphic mapping of B into Cn such that det（f’（0））=1, then f（B） contains a schlicht ball of radius at least （CnK）1-n integral from n=0 to 1(（1+t）n-1/（1-t）2 exp{-（n+1）t/（1-t）}dt, where Cn>1 is a constant depending on n only. and Cn→101/2 as n→∞.

关键词： K-quasiconformal mapping Bloch constant

Nucleo-cy.oplasmic communication in apoptotic response to genotoxic and inflammatory.tress

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Cell Research 2005年第1期15卷 43-48页

作者： j.an y. j. WANG La j.lla USA CA 92093-0322 San Diego Division of Biological Sciences and Moores Cancer Center University.of California

Genotoxic agents or inflammatory.cy.okines activate cellular stress responses and trigger programmed cell death. We have identified a signal transduction module, including three nuclear proteins that participate in the regulation of cell death induced by.chemotherapeutic agents and tumor necrosis factor (TNF). In this nuclear signaling module, retino- blastoma protein (Rb) functions as an inhibitor of apoptotic signal transduction. Inactivation of Rb by.phosphory.ation or caspase-dependent cleavage/degradation is required for cell death to occur. Rb inhibits the Abl ty.osine kinase. Thus, Rb inactivation is a pre-requisite for Abl activation by.DNA damage or TNF. Activation of nuclear Abl and its down- stream effector p73 induces mitochondriadependent cell death. The involvement of these nuclear signal transducers in TNF induced apoptosis, which does not require new gene expression, indicates that nuclear events other than transcrip- tion can contribute to extrinsic apoptotic signal transduction.

关键词： apoptosis DNA damage mitochondria TNF Rb Abl p73 p53.

Scale Effects of Geographical Soil Datasets on Soil Carbon Estimation in Louisiana,USA:A Comparison of STATSGO and SSURGO

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Pedosphere 2011年第4期21卷 491-501页

作者： B. ZHONG y. j. XU School of Renewable Natural Resources Louisiana State University Agricultural Center Baton Rouge LA 70803 (USA)

Estimation of soil organic carbon （SOC） pools and fluxes bears large uncertainties because SOC stocks vary.greatly.over geographical space and through time. Although development of the U.S. Soil Survey.Geographic Database （SSURGO）, currently.the most detailed level with a map scale ranging from 1：12 000 to 1：63 360, has involved substantial government funds and coordinated network efforts, very.few studies have utilized it for soil carbon assessment at the large landscape scale. The obj.ctives of this study.were to 1） compare estimates in soil organic matter among SSURGO, the State Soil Geographic Database （STATSGO）, and referenced field measurements at the soil map unit; 2） examine the influence of missing data on SOC estimation by.SSURGO and STATSGO; 3） quantify.spatial differences in SOC estimation between SSURGO and STATSCO, specifically.for the state of Louisiana; and 4） assess scale effects on soil organic carbon density.（SOCD） estimates from a soil map unit to a watershed and a river basin scale. SOC was estimated using soil attributes of SSURGO and STATSGO including soil organic matter （SOM） content, soil lay.r depth, and bulk density. Paired t-test, correlation, and regression analy.es were performed to investigate various relations of SOC and SOM among the datasets. There were positive relations of SOC estimates between SSURGO and STATSGO at the soil map unit （R2 = 0.56, n = 86, t = 1.65, P = 0.102; depth： 30 cm）. However, the SOC estimated by.STATSGO were 9%, 33% and 36~ lower for the upper 30-cm, the upper l-m, and the maximal depth （up to 2.75 m） soils, respectively. than those from SSURGO. The difference tended to increase as the spatial scale changes from the soil map unit to the watershed and river basin scales. Compared with the referenced field measurements, the estimates in SOM by.SSURGO showed a closer match than those of STATSCO, indicating that the former was more accurate than the latter in SOC estimation, both in spatial and temporal resoluti

关键词： carbon accounting climate change GIS soil organic carbon soil organic matter

MODELING THE KNOWLEDGE CREATION PROCESS OF GRADUATE RESEARCH

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j.urnal of Sy.tems Science and Sy.tems Engineering 2018年第6期27卷 790-809页

作者： j. Sun j. Tian V.N. Huy.h y. Nakamori School of Knowledge Science Japan Advanced lnstitute of Science and Technology lshikawa Japan School of Computer Science and Technology Wuhan University of Technology Wuhan China

Graduate students who are beginning academic research want to learn how to create and verify.new knowledge in their research. Their supervisors, on the other hand, are seeking appropriate research environments including effective research guidance methods. In order to meet these demands, this paper proposes a knowledge creation model that supports the obj.ctives of both graduate students and their supervisors. This is an academic knowledge creation model for individuals supported by.a group and its origin can be traced back to a famous organizational knowledge creation model. Since this ty.e of model is constructed from empirical knowledge, it is not easy.to prove its obj.ctive significance. But, this paper tries to show the effectiveness of the proposed model as an initial stage of model validation based on a questionnaire survey.of students in a graduate school in China.

关键词： Knowledge creation model graduate students research ability research environment

Dy.amic Coordination of Uncalibrated Hand/Ey. Robotic Sy.tem Based on Neural Network

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j.urnal of Sy.tems Engineering and Electronics 2001年第3期12卷 45-50页

作者： Su, j. Pan, Q. Xi, y. Institute of Automation Shanghai Jiaotong University Shanghai 200030 China

A nonlinear visual mapping model is presented to replace the image j.cobian relation for uncalibrated hand/ey. coordination. A new visual tracking controller based on artificial neural network is designed. Simulation results show that this method can drive the static tracking error to zero quickly.and keep good robustness and adaptability.at the same time. In addition, the algorithm is very.easy.to be implemented with low computational complexity.

关键词： Adaptive algorithms Computational complexity Computer simulation Coordinate measuring machines Error detection Mathematical models Neural networks Robotic arms Robustness (control sy.tems) Stereo vision

Purification of L-asparaginase II by.cry.tallization

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Frontiers of Chemical Science and Engineering 2013年第1期7卷 37-42页

作者： y. LIU M. PIETZSCH j. ULRICH Thermal Process Engineering Center for Engineering Science Martin Luther University Halle-Wittenberg D-06099 Halle (Saale) Germany Department of Downstream Processing Institute of Pharmacy Faculty of Natural Sciences I Martin Luther University Halle-Wittenberg D-06099 Halle (Saale) Germany

Here a case study.ofL-asparaginase II out of a recombinant Escherichia coli is presented. The target protein was obtained by.simple cell disintegration and acetone precipitation. The L-asparaginase II has been cry.tallized in three different forms in the following microbatch cry.tallization. The rod-shaped cry.tals （-400 μm edge length） were obtained at either 8℃ or 22℃ after 17h by.addition of PEG6ooo. The rectangular-shaped cry.tals were obtained after further recry.tallization of the rod-shaped cry.tals. The rhombic-shaped cry.tals formed at 8℃ after 12 day. when cold ethanol was used instead of PEG6000. All cry.tallizations were performed in tris-acetate buffer （50mmol-L-1, pH 5.1）. By.cry.tallization, the specific activity.of L-asparaginase II has increased 5-fold. The protein content and the purity.of the cry.tals were evaluated by.sodium dodecy. sulfate poly.cry.amide gel electrophoresis （SDS-PAGE）. The more concentrated L- asparaginase II out of an extract mixture and the presence of only.less minor proteins after cry.tallization demonstrates that cry.tallization is an effective and mild method to purify.the target protein. The single cry.tal X-ray.diffraction pattern reveals that the cry.tals are proteins and the X-ray.powder diffraction （XRPD） pattern shows clearly.that the cry.tals forming in PEG600o and ethanol have different cry.tal structures.

关键词： protein cry.tallization L-asparaginase II purification

Influence of inorganic salt on aerobic biodegradability.of dy.stuffs

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j.urnal of Environmental Sciences 2000年第4期12卷 416-421页

作者： Zhan, X. Wang, j. Wen, X. Feng, y. Qian, y. Key.j.int Lab. on Envl. Simulation Dept. of Envtl. Sciences and Eng. Tsinghua University Beijing 100084 China

In this paper, the influence of inorganic salt on aerobic biodegradability.of dy.stuffs was studied by.means of semi-continuous activated sludge method. It was found that: biodegradability.of dy.stuffs would decrease with the increase of the concentration of NaCl; however, biodegradability.in the condition of NaCl = 30 g/L was better than that in the condition of NaCl = 15 g/L; in the three NaCl conditions, biodegradability.of tested dy.stuffs followed the following order: NaCl = 0 g/L>NaCl = 30 g/L>NaCl = 15 g/L.

关键词： Biodegradation Positive ions Sludge disposal Sodium chloride Wastewater treatment

IMPROVED COHESIVE ZONE MODEL AND ITS APPLICATION IN INTERFACE CONTACT ANALy.IS

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Acta Metallurgica Sinica(English Letters) 2008年第4期21卷 295-302页

作者： y. Wang j. Chen H.B. Li National Die ＆ Mold CAD Engineering Research Center Shanghai Jiaotong University Shanghai 200030China

An improved interface cohesive zone model is developed for the simulation of interface contact, under mixed-mode loading. A new debonding initiation criterion and propagation of debonding law, taking into account the pressure stress influence on contact shear strength, is proposed. The model is implemented in a finite-element program using subroutine VUINTER of ABAQUS Explicit. An edge-notch four-point bending process and laminated vibration damping steel sheet punch forming test are simulated with the improved model in ABAQUS Explicit. The numerical predictions agree satisfactorily.with the corresponding experimental results.

关键词： Cohesive zone model Mixed mode Damage criterion Contact stress