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Gene therapy for spinal muscular atrophy:perspectives on the possibility of optimizing SMN1 delivery to correct all neurological and systemic 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.Yáñez-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 symptom onset and developmental milestones achieved,with life expectancy and severity varying between ***,SMN2 copy number is used as a proxy for the prediction of disease severity,with higher SMN2 copy number typically being associated with reduced severity of SMA,although this relationship is not absolute:some individuals with low SMN2 copy number have less severe SMA phenotypes and vice ***,the etiology of SMA is further complicated by other factors,such as non-typical nucleotide variants and SMN2-independent modifiers of disease severity.

关键词： atrophy traditionally absolute

基于泊肃叶定律的毛细管型血液粘度计工作原理的物理模拟实验研究

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伊犁师范学院学报（自然科学版） 2017年第3期11卷 62-64页

作者：刘慧余蓉敬小丽王旭东高丽霞木本荣成都中医药大学医学技术学院四川成都610000

根据泊肃叶定律,采用不同浓度的甘油模拟不同血浆浓度,通过毛细管粘度计对甘油粘度系数进行测定.根据毛细管粘度计的实验数据,总结出毛细管粘度计的工作原理,并确定甘油粘度与浓度的关系,以探索毛细管粘度计在血液粘度测量中的适用范围.

关键词：毛细管粘度计甘油物理模拟

Müller cells are activated in response to retinal outer nuclear layer degeneration in rats subjected to simulated weightlessness conditions

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Neural Regeneration Research 2025年第7期20卷 2116-2128页

作者： Yuxue mu Ning Zhang Dongyu Wei Guoqing Yang Lilingxuan Yao Xinyue Xu Yang Li Junhui Xue Zuoming Zhang Tao Chen Aerospace Clinical Medical Center School of Aerospace MedicineAir Force Medical UniversityXi’anShaanxi ProvinceChina Department of Aviation Medicine Xijing HospitalAir Force Medical UniversityXi’anShaanxi ProvinceChina Department of Emergency Medicine Wuhan No.1 HospitalWuhanHubei ProvinceChina Third Regiment School of Basic MedicineAir Force Medical UniversityXi’anShaanxi ProvinceChina Fourth Regiment School of Basic MedicineAir Force Medical UniversityXi’anShaanxi ProvinceChina

A microgravity environment has been shown to cause ocular damage and affect visual acuity,but the underlying mechanisms remain ***,we established an animal model of weightlessness via tail suspension to examine the pathological changes and molecular mechanisms of retinal damage under *** 4 weeks of tail suspension,there were no notable alterations in retinal function and morphology,while after 8 weeks of tail suspension,significant reductions in retinal function were observed,and the outer nuclear layer was thinner,with abundant apoptotic *** investigate the mechanism underlying the degenerative changes that occurred in the outer nuclear layer of the retina,proteomics was used to analyze differentially expressed proteins in rat retinas after 8 weeks of tail *** results showed that the expression levels of fibroblast growth factor 2(also known as basic fibroblast growth factor)and glial fibrillary acidic protein,which are closely related to Müller cell activation,were significantly *** addition,Müller cell regeneration and Müller cell gliosis were observed after 4 and 8 weeks,respectively,of simulated *** findings indicate that Müller cells play an important regulatory role in retinal outer nuclear layer degeneration during weightlessness.

关键词： glial fibrous acidic protein gliosis Müller cells nerve growth factor neural differentiation neurodegeneration proteomic retinal degeneration retinal outer nuclear layer simulated weightlessness

EZH2-dependent myelination following sciatic nerve injury

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Neural Regeneration Research 2025年第8期20卷 2382-2394页

作者： Hui Zhu Li mu Xi Xu Tianyi Huang Ying Wang Siyuan Xu Yiting Wang Wencong Wang Zhiping Wang Hongkui Wang Chengbin Xue Research Center of Clinical Medicine Affiliated Hospital of Nantong UniversityKey Laboratory of Neuroregeneration of Jiangsu and Ministry of EducationCo-innovation Center of NeuroregenerationNMPA Key Laboratory for Research and Evaluation of Tissue Engineering Technology ProductsNantong UniversityNantongJiangsu ProvinceChina Medical School of Nantong University NantongJiangsu ProvinceChina Department of Neurology Affiliated Hospital of Nantong UniversityNantongJiangsu ProvinceChina Department of Rehabilitation Medicine Affiliated Hospital of Nantong UniversityNantongJiangsu ProvinceChina Department of Critical Care Medicine Nantong Fourth People’s HospitalNantongJiangsu ProvinceChina

Demyelination and remyelination have been major focal points in the study of peripheral nerve regeneration following peripheral nerve ***,the gene regulatory network of regenerated myelin differs from that of native *** of enhancer of zeste homolog 2(EZH2)hinders the differentiation,maturation,and myelination of Schwann cells in *** further determine the role of EZH2 in myelination and recovery post-peripheral nerve injury,conditional knockout mice lacking Ezh2 in Schwann cells(Ezh2^(fl/fl);Dhh-Cre and Ezh2^(fl/fl);Mpz-Cre)were *** results show that a significant proportion of axons in the sciatic nerve of Ezh2-depleted mice remain *** highlights the crucial role of Ezh2 in initiating Schwann cell ***,we observed that 21 days after inducing a sciatic nerve crush injury in these mice,most axons had remyelinated at the injury site in the control nerve,while Ezh2^(fl/fl);Mpz-Cre mice had significantly fewer remyelinated axons compared with their wild-type *** suggests that the absence of Ezh2 in Schwann cells impairs myelin formation and *** conclusion,EZH2 has emerged as a pivotal regulatory factor in the process of demyelination and myelin regeneration following peripheral nerve *** EZH2 activity during these processes may offer a promising therapeutic target for the treatment of peripheral nerve injuries.

关键词： demyelination EZH2 myelination peripheral nerve injury PRC2 remyelination Schwann cells sciatic nerve crush sciatic nerve transection

CNKSR2 interactome analysis indicates its association with the centrosome/microtubule system

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Neural Regeneration Research 2025年第8期20卷 2420-2432页

作者： Lin Yin Yalan Xu Jie mu Yu Leng Lei Ma Yu Zheng Ruizhi Li Yin Wang Peifeng Li Hai Zhu Dong Wang Jing Li Institute for Translational Medicine The Affiliated Hospital of Qingdao UniversityMedical CollegeQingdao UniversityQingdaoShandong ProvinceChina School of Basic Medicine Qingdao UniversityQingdaoShandong ProvinceChina College of Life Sciences and School of PharmacyMedical CollegeQingdao UniversityQingdaoShandong ProvinceChina Department of Urology Qingdao Municipal Hospital Affiliated to Qingdao UniversityQingdaoShandong ProvinceChina

The protein connector enhancer of kinase suppressor of Ras 2(CNKSR2),present in both the postsynaptic density and cytoplasm of neurons,is a scaffolding protein with several protein-binding *** of the CNKSR2 gene have been implicated in neurodevelopmental disorders,particularly intellectual disability,although the precise mechanism involved has not yet been fully *** has demonstrated that CNKSR2 plays a role in facilitating the localization of postsynaptic density protein complexes to the membrane,thereby influencing synaptic signaling and the morphogenesis of dendritic ***,the function of CNKSR2 in the cytoplasm remains to be *** this study,we used immunoprecipitation and high-resolution liquid chromatography-mass spectrometry to identify the interactors of *** a combination of bioinformatic analysis and cytological experiments,we found that the CNKSR2 interactors were significantly enriched in the proteome of the *** also showed that CNKSR2 interacted with the microtubule protein DYNC1H1 and with the centrosome marker *** colocalization analysis confirmed the centrosomal localization of *** we downregulated CNKSR2 expression in mouse neuroblastoma cells(Neuro 2A),we observed significant changes in the expression of numerous centrosomal *** manipulation also affected centrosome-related functions,including cell size and shape,cell proliferation,and ***,we found that CNKSR2 interactors were highly enriched in de novo variants associated with intellectual disability and autism spectrum *** findings establish a connection between CNKSR2 and the centrosome,and offer new insights into the underlying mechanisms of neurodevelopmental disorders.

关键词： autism spectrum disorder centrosome CNKSR2 intellectual disability interactome mass spectrometry microtubule neurodevelopmental disease protein complexes protein-protein interactions

Seismic attenuation relationship with homogeneous and heterogeneous prediction-error variance models

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Earthquake Engineering and Engineering Vibration 2014年第1期13卷 1-11页

作者： He-Qing mu rong-rong Xu Ka-Veng Yuen Faculty of Science and Technology University of Macao

Peak ground acceleration(PGA) estimation is an important task in earthquake engineering *** of the most well-known models is the Boore-Joyner-Fumal formula,which estimates the PGA using the moment magnitude,the site-to-fault distance and the site foundation *** the present study,the complexity for this formula and the homogeneity assumption for the prediction-error variance are investigated and an effi ciency-robustness balanced formula is *** this purpose,a reduced-order Monte Carlo simulation algorithm for Bayesian model class selection is presented to obtain the most suitable predictive formula and prediction-error model for the seismic attenuation *** this approach,each model class(a predictive formula with a prediction-error model) is evaluated according to its plausibility given the *** one with the highest plausibility is robust since it possesses the optimal balance between the data fi tting capability and the sensitivity to noise.A database of strong ground motion records in the Tangshan region of China is obtained from the China Earthquake Data Center for the *** optimal predictive formula is proposed based on this *** is shown that the proposed formula with heterogeneous prediction-error variance is much simpler than the attenuation model suggested by Boore,Joyner and Fumal(1993).

关键词： Bayesian inference Boore-Joyner-Fumal formula heterogeneity variance input-dependent variance model class selection peak ground acceleration seismic attenuation

ATTRACTORS FOR DISCRETIZATION OF GINZBURG-LANDAU-BBM EQUATIONS

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Journal of Computational Mathematics 2001年第2期19卷 195-204页

作者： mu-rong Jiang Bo-ling Guo Department of Mathematics Yunnan UniversityKunming 650091China R&D Center for Parallel Software Institute of SoftwareChinese Academy of SciencesBeijing 100080China Institute of Applied Physics and Computational Mathematics Beijing 100088China

Focuses on a study which discreted Ginzburg-Landau-BBM equations with periodic initial boundary value conditions by the finite difference method in spatial direction. Background on the discretization of the equations and the priori estimates; Existence of the attractors for the discrete system; Estimates of the upper bounds of Hausdorff and fractal dimensions for the attractors.

关键词： attractor spatially discreted Ginzburg-Landau-BBM equations Hausdorff and fractal dimensions

Influence of Positive and Negative Indian Ocean Dipoles on ENSO via the Indonesian Throughflow: Results from Sensitivity Experiments

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Advances in Atmospheric Sciences 2015年第6期32卷 783-793页

作者： ZHOU Qian DUAN Wansuo mu mu FENG rong State Key Laboratory of Numerical Modeling for Atmospheric Sciences and Geophysical Fluid Dynamics Institute of Atmospheric Physics Chinese Academy of Sciences University of Chinese Academy of Sciences Key Laboratory of Ocean Circulation and Wave Institute of Oceanology Chinese Academy of Sciences

The role of the Indonesian Throughflow （ITF） in the influence of the Indian Ocean Dipole （IOD） on ENSO is investigated using version 2 of the Parallel Ocean Program （POP2） ocean general circulation model. We demonstrate the results through sensitivity experiments on both positive and negative IOD events from observations and coupled general circulation model simulations. By shutting down the atmospheric bridge while maintaining the tropical oceanic channel, the IOD forcing is shown to influence the ENSO event in the following year, and the role of the ITF is emphasized. During positive IOD events, negative sea surface height anomalies （SSHAs） occur in the eastern Indian Ocean, indicating the existence of upwelling. These upwelling anomalies pass through the Indonesian seas and enter the western tropical Pacific, resulting in cold anomalies there. These cold temperature anomalies further propagate to the eastern equatorial Pacific, and ultimately induce a La Nifia- like mode in the following year. In contrast, during negative IOD events, positive SSHAs are established in the eastern Indian Ocean, leading to downwelling anomalies that can also propagate into the subsurface of the western Pacific Ocean and travel further eastward. These downwelling anomalies induce negative ITF transport anomalies, and an E1 Nifio-like mode in the tropical eastern Pacific Ocean that persists into the following year. The effects of negative and positive IOD events on ENSO via the ITF are symmetric. Finally, we also estimate the contribution of IOD forcing in explaining the Pacific variability associated with ENSO via ITE

关键词： IOD Pacific Ocean ENSO Indonesian Throughflow

Energy of Long-Lifetime Configurations in Zero-Temperature Dynamics

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Communications in Theoretical Physics 2009年第4期51卷 671-674页

作者： rong Yong-Hui KONG Xiang-mu College of Physics and Engineering Qufu Normal University Qufu 273165 China

Using Monte Carlo method with zero-temperature dynamics, we investigate energy evolution of Ising spin configuration on a square lattice. The energies of some configurations exhibit long duration before those configurations reach the final state -- ground state or frozen stripe state. For ground-state dynamical realization, the duration occurs when the energy per spin is 4/L, where L is the lattice size. For stripe-state dynamical realization, the energy is slightly higher than 2/L when the duration appears in the last evolution stage. In addition, it is found that the average energy per spin in final state is approximately 2/3L.

关键词： Ising model zero-temperature dynamics Monte Carlo method