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A novel mutation of RPGR in a Chinese family with X-linked retinitis pigmentosa

International Journal of Ophthalmology(English edition) 2022年第9期15卷 1423-1430页

作者： Hui-Hui sun Jing-Cong Zhao su-ling yang Jin-Dou Shi Yun-Shuo Wei Jian-Cang Wang Feng Gu Lu Chen Children's Hospital of Hebei Province Shijiazhuang 0050000Hebei ProvinceChina Hebei General Hospital Shijiazhuang 050000Hebei ProvinceChina School of Ophthalmology and Optometry Eye HospitalWenzhou Medical UniversityState Key Laboratory Cultivation Base and Key Laboratory of Vision ScienceMinistry of Health and Zhejiang Provincial Key Laboratory of Ophthalmology and OptometryWenzhou 325027Zhejiang ProvinceChina

·AIM:To identify potential mutations and elucidate the clinical findings of male patients and female carriers of X-Iinked retinitis pigmentosa(XLRP)in a Chinese family.·METHODS:A four generation pedigree was collected that consisted of 20 *** DNA was extracted from peripheral blood,and then the target fragments were amplified by PCR and sequenced *** addition,all affected patients and female carriers underwent comprehensively ophthalmic evaluation.·REsuLTS:A novel mutation c.2865 G>A p.W955 X in RPGR gene was identified of this family,including four affected individuals and eight *** male patients,aging from 7 to 31 y,tended to have more various,even potentially deleterious clinical features of *** the same time,individuals with heterozygous mutations(carriers)manifested a wide spectrum of clinical ***,only two male patients and three female carriers manifested pathological myopia(PM).Among the female carriers,half of subjects who harbor poor visual acuity suffered esotropia or ***,16.7%and 66.7%of carriers had abnormal electroretinogram(ERG)and fundus,respectively.·CONCLUSION:In this study,a novel mutation of the RPGR gene is identified,which broadens the spectrum of RPGR mutations,and elaborates the relationship between genotype and phenotype.

关键词： X-linked retinitis pigmentosa RPGR nonsense mutation phenotype

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A surface engineering strategy for the stabilization of zinc metal anodes with montmorillonite layers toward long-life rechargeable aqueous zinc ion batteries

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Journal of Energy Chemistry 2025年第1期100卷 94-105页

作者： Wenbo Wang Ruifeng Xu Xu Zhang Peiyu Wang Bao yang Bingjun yang Juan yang Kailimai su Pengjun Ma Yanan Deng Xianfeng Fan Wanjun Chen Key Laboratory for Electronic Materials College of Electrical Engineering Northwest Minzu University Resource Chemistry and Energy Materials Research Center State Key Laboratory of Solid Lubrication Lanzhou Institute of Chemical Physics Chinese Academy of Sciences University of Chinese Academy of Sciences Institute for Materials and Processes School of Engineering University of Edinburgh

Rechargeable aqueous zinc-ion batteries （AZIBs） exhibit appreciable potential in the domain of electrochemical energy ***,there are serious challenges for AZIBs,for instance zinc dendrite growth,hydrogen evolution reaction （HER）,and corrosion side ***,we propose a surface engineering modification strategy for coating the montmorillonite （MMT） layer onto the surface of the Zn anode to tackle these issues,thereby achieving high cycling stability for rechargeable *** results reveal that the MMT layer on the surface of the Zn anode is able to provide ordered zincophilic channels for zinc ions migration,facilitating the reaction kinetics of zinc *** functional theory（DFT） calculations and water contact angle （CA） tests prove that MMT@Zn anode exhibits superior adsorption capacity for Zn2+and better hydrophobicity than the bare Zn anode,thereby achieving excellent cycling ***,the MMT@Zn||MMT@Zn symmetric cell holds the stable cycling over 5600 h at 0.5 mA cm-2and 0.125 m A h cm-2,even exceeding 1800 h long cycling under harsh conditions of 5 m A cm-2and 1.25 m A h *** MMT@Zn||V2O5full cell reaches over 3000 cycles at 2 A g-1with excellent rate ***,this surface engineering modification strategy for enhancing the electrochemical performance of AZIBs represents a promising application.

关键词： surface engineering strategy Montmorillonite layer Protection mechanism of Zn anode Cycling stability Aqueous zinc ion batteries

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乡镇企业的发展与新农村生态环境改善——以温州为例

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浙江工贸职业技术学院学报 2013年第3期13卷 22-25页

作者：凌苏阳吉林大学军需科技学院吉林长春130012

在社会主义新农村建设推进过程中,乡镇企业不断地发展,为农村经济的增长做出了巨大贡献,但随之而来的环境污染问题给生态环境造成了巨大压力,对农村社会事业的建设和农民生产生活质量的提高产生了重大影响。农村生态环境的改善是新农村... 详细信息

在社会主义新农村建设推进过程中,乡镇企业不断地发展,为农村经济的增长做出了巨大贡献,但随之而来的环境污染问题给生态环境造成了巨大压力,对农村社会事业的建设和农民生产生活质量的提高产生了重大影响。农村生态环境的改善是新农村建设的重要方面。本文就温州乡镇企业的发展对环境造成的破坏进行了详细分析,并就如何保护农村生态环境,实施可持续发展提出对策。

关键词：温州乡镇企业新农村生态环境

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Nanoparticles for the treatment of spinal cord injury

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Neural Regeneration Research 2025年第6期20卷 1665-1680页

作者： Qiwei yang Di Lu Jiuping Wu Fuming Liang Huayi Wang Junjie yang Ganggang Zhang Chen Wang Yanlian yang ling Zhu Xinzhi sun Department of Orthopedic surgery The First Affiliated Hospital of Zhengzhou UniversityZhengzhouHenan ProvinceChina CAS Key Laboratory of Standardization and Measurement for Nanotechnology CAS Key Laboratory for Biomedical Effects of Nanomaterials and NanosafetyCAS Center for Excellence in NanoscienceNational Center for Nanoscience and TechnologyBeijingChina University of Chinese Academy of Sciences BeijingChina Department of Neurosurgery The First Affiliated Hospital of Chongqing Medical UniversityChongqingChina

Spinal cord injuries lead to significant loss of motor, sensory, and autonomic functions, presenting major challenges in neural regeneration. Achieving effective therapeutic concentrations at injury sites has been a slow process, partly due to the difficulty of delivering drugs effectively. Nanoparticles, with their targeted delivery capabilities, biocompatibility, and enhanced bioavailability over conventional drugs, are garnering attention for spinal cord injury treatment. This review explores the current mechanisms and shortcomings of existing treatments, highlighting the benefits and progress of nanoparticle-based approaches. We detail nanoparticle delivery methods for spinal cord injury, including local and intravenous injections, oral delivery, and biomaterial-assisted implantation, alongside strategies such as drug loading and surface modification. The discussion extends to how nanoparticles aid in reducing oxidative stress, dampening inflammation, fostering neural regeneration, and promoting angiogenesis. We summarize the use of various types of nanoparticles for treating spinal cord injuries, including metallic, polymeric, protein-based, inorganic non-metallic, and lipid nanoparticles. We also discuss the challenges faced, such as biosafety, effectiveness in humans, precise dosage control, standardization of production and characterization, immune responses, and targeted delivery in vivo. Additionally, we explore future directions, such as improving biosafety, standardizing manufacturing and characterization processes, and advancing human trials. Nanoparticles have shown considerable progress in targeted delivery and enhancing treatment efficacy for spinal cord injuries, presenting significant potential for clinical use and drug development.

关键词： antioxidants axon regeneration biocompatible materials drug carriers nanoparticles nerve regeneration neuroinflammatory diseases neuroprotection spinal cord injury stem cells

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Deep eutectic solvents for separation and purification applications in critical metal metallurgy:Recent advances and perspectives

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International Journal of Minerals,Metallurgy and Materials 2025年第1期32卷 1-19页

作者： Shuo Chen Shengpeng su Yanfang Huang Bingbing Liu Hu sun Shuzhen yang Guihong Han School of Chemical Engineering Zhengzhou UniversityZhengzhou 450001China Zhongyuan Critical Metals Laboratory Henan ProvinceZhengzhou 450001China

Solvent extraction,a separation and purification technology,is crucial in critical metal *** solvents commonly used in solvent extraction exhibit disadvantages,such as high volatility,high toxicity,and flammability,causing a spectrum of hazards to human health and environmental *** solvents have been recognized as potential alternatives to these harmful organic *** the past two decades,several neoteric solvents have been proposed,including ionic liquids(ILs)and deep eutectic solvents(DESs).DESs have gradually become the focus of green solvents owing to several advantages,namely,low toxicity,degradability,and low *** this critical review,their classification,formation mechanisms,preparation methods,characterization technologies,and special physicochemical properties based on the most recent advancements in research have been systematically ***,the major separation and purification applications of DESs in critical metal metallurgy were comprehensively ***,future opportunities and challenges of DESs were explored in the current research *** conclusion,this review provides valuable insights for improving our overall understanding of DESs,and it holds important potential for expanding separation and purification applications in critical metal metallurgy.

关键词： deep eutectic solvents preparations properties separation and purification critical metal metallurgy

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Progress of research in the application of ultrasound technology for the treatment of Alzheimer’s disease

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Neural Regeneration Research 2025年第10期20卷 2823-2837页

作者： Qiuquan Cai Lianghui Meng Meina Quan ling Wang Jing Ren Chenguang Zheng Jiajia yang Dong Ming Academy of Medical Engineering and Translational Medicine Tianjin UniversityTianjinChina Innovation Center for Neurological Disorders Xuanwu HospitalCapital Medical UniversityBeijingChina Brain-Computer Interaction and Human-Machine Fusion Haihe Laboratory Tianjin UniversityTianjinChina Tianjin Key Laboratory of Brain Science and Neural Engineering Tianjin UniversityTianjinChina

Alzheimer’s disease is a common neurodegenerative disorder defined by decreased reasoning abilities,memory loss,and cognitive *** presence of the blood-brain barrier presents a major obstacle to the development of effective drug therapies for Alzheimer’s *** use of ultrasound as a novel physical modulation approach has garnered widespread attention in recent *** a safe and feasible therapeutic and drug-delivery method,ultrasound has shown promise in improving cognitive *** article provides a summary of the application of ultrasound technology for treating Alzheimer’s disease over the past 5 years,including standalone ultrasound treatment,ultrasound combined with microbubbles or drug therapy,and magnetic resonance imaging-guided focused ultrasound *** is placed on the benefits of introducing these treatment methods and their potential *** found that several ultrasound methods can open the blood-brain barrier and effectively alleviate amyloid-βplaque *** believe that ultrasound is an effective therapy for Alzheimer’s disease,and this review provides a theoretical basis for future ultrasound treatment methods.

关键词： Alzheimer’s disease blood-brain barrier drugs magnetic resonance imaging-guided focused ultrasound microbubbles scanning ultrasound ultrasound ultrasound stimulation

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Gamma-glutamyl transferase 5 overexpression in cerebrovascular endothelial cells improves brain pathology,cognition,and behavior in APP/PS1 mice

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Neural Regeneration Research 2025年第2期20卷 533-547页

作者： Yanli Zhang Tian Li Jie Miao Zhina Zhang Mingxuan yang Zhuoran Wang Bo yang Jiawei Zhang Haiting Li Qiang su Junhong Guo Department of Neurology First Hospital of Shanxi Medical UniversityTaiyuanShanxi ProvinceChina Department of Neurology Sixth Hospital of Shanxi Medical University(General Hospital of Tisco)TaiyuanShanxi ProvinceChina Department of Physiology Key Laboratory of Cellular PhysiologyMinistry of EducationShanxi Medical UniversityTaiyuanShanxi ProvinceChina Department of Hernia and Abdominal Wall surgery Shanxi Bethune HospitalShanxi Academy of Medical SciencesTongji Shanxi HospitalThird Hospital of Shanxi Medical UniversityTaiyuanShanxi ProvinceChina Department of Laboratory Medicine of Fenyang College Shanxi Medical UniversityFenyangShanxi ProvinceChina

In patients with Alzheimer’s disease,gamma-glutamyl transferase 5(GGT5)expression has been observed to be downregulated in cerebrovascular endothelial ***,the functional role of GGT5 in the development of Alzheimer’s disease remains *** study aimed to explore the effect of GGT5 on cognitive function and brain pathology in an APP/PS1 mouse model of Alzheimer’s disease,as well as the underlying *** observed a significant reduction in GGT5 expression in two in vitro models of Alzheimer’s disease(Aβ_(1-42)-treated hCMEC/D3 and bEnd.3 cells),as well as in the APP/PS1 mouse ***,injection of APP/PS1 mice with an adeno-associated virus encoding GGT5 enhanced hippocampal synaptic plasticity and mitigated cognitive ***,increasing GGT5 expression in cerebrovascular endothelial cells reduced levels of both soluble and insoluble amyloid-βin the brains of APP/PS1 *** effect may be attributable to inhibition of the expression ofβ-site APP cleaving enzyme 1,which is mediated by nuclear factor-kappa *** findings demonstrate that GGT5 expression in cerebrovascular endothelial cells is inversely associated with Alzheimer’s disease pathogenesis,and that GGT5 upregulation mitigates cognitive deficits in APP/PS1 *** findings suggest that GGT5 expression in cerebrovascular endothelial cells is a potential therapeutic target and biomarker for Alzheimer’s disease.

关键词： Alzheimer’s disease amyloid-β APP/PS1 mice cerebrovascular endothelial cells cognitive deficits gamma-glutamyl transferase 5 neurovascular unit nuclear factor‐kappa B synaptic plasticity β-site APP cleaving enzyme 1

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Bullous pemphigoid associated with acquired hemophilia A: A case report

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World Journal of Clinical Cases 2025年第4期13卷 28-33页

作者： su-Ye Hu Meng-Can Li Zi-Jia Hao Xu-Ya Chai Pei-Sai Li yang Liu Li-Xia Liu Ying Xu Pan-Pan yang ling-E Li Department of Dermatology Shijiazhuang Traditional Chinese Medicine HospitalShijiazhuang 050051Hebei ProvinceChina Graduate School Hebei University of Traditional Chinese MedicineShijiazhuang 050051Hebei ProvinceChina Department of Pathology Shijiazhuang Traditional Chinese Medicine HospitalShijiazhuang 050051Hebei ProvinceChina

BACKGROUND Acquired hemophilia A (AHA) is a rare and potentially severe bleeding disordercaused by circulating autoantibodies against factor Ⅷ (FⅧ). In approximately50% of the patients, the condition is associated with autoimmune diseases,cancers, medication use, pregnancy, and the post-partum period. Bullous pemphigoid(BP) is a chronic autoimmune subepidermal blistering disease associatedwith tissue-bound and circulating autoantibodies against BP antigens 180 (BP180)and 230 (BP230). AHA-associated BP has a high mortality rate;hence, the understandingof this disease must *** suMMARY A 69-year-old man presented with erythema, blisters, blood blisters, and crustsaccompanied by severe pruritus for more than 20 days, and ecchymosis andswelling on his left upper arm for 3 days. Pathological examination revealed asubepidermal blister that contained eosinophils. Laboratory tests showed that theBP180 autoantibody levels had increased, isolated activated partial thromboplastintime was notably prolonged (115.6 s), and coagulation FⅧ activity wasextremely low (< 1.0%). Furthermore, the FⅧ inhibitor titer had greatlyincreased (59.2 Bethesda units). Therefore, the patient was diagnosed as having BP associated with AHA, prescribed 0.05% topical halometasone cream, and transferred to a higher-level hospitalfor effective treatment;however, he died after 2 *** AHA associated BP is rare, dangerous, and has a high mortality rate. Therefore, its timely diagnosis and effectivetreatment are necessary.

关键词： Bullous pemphigoid Acquired hemophilia A Acquired hemophilia A FactorⅧ Case report

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Reduced mesencephalic astrocyte-derived neurotrophic factor expression by mutant androgen receptor contributes to neurodegeneration in a model of spinal and bulbar muscular atrophy pathology

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Neural Regeneration Research 2025年第9期20卷 2655-2666页

作者： Yiyang Qin Wenzhen Zhu Tingting Guo Yiran Zhang Tingting Xing Peng Yin Shihua Li Xiao-Jiang Li su yang Guangdong Key Laboratory of Non-human Primate Research Key Laboratory of CNS Regeneration(Ministry of Education)Guangdong–HongKong–Macao Institute of CNS RegenerationJinan UniversityGuangzhouGuangdong ProvinceChina

Spinal and bulbar muscular atrophy is a neurodegenerative disease caused by extended CAG trinucleotide repeats in the androgen receptor gene,which encodes a ligand-dependent transcription facto *** mutant androgen receptor protein,characterized by polyglutamine expansion,is prone to misfolding and forms aggregates in both the nucleus and cytoplasm in the brain in spinal and bulbar muscular atrophy *** aggregates alter protein-protein interactions and compromise transcriptional *** this study,we reported that in both cultured N2a cells and mouse brain,mutant androgen receptor with polyglutamine expansion causes reduced expression of mesencephalic astrocyte-de rived neurotrophic *** n of mesencephalic astrocyte-derived neurotrophic factor amelio rated the neurotoxicity of mutant androgen receptor through the inhibition of mutant androgen receptor *** down endogenous mesencephalic astrocyte-derived neurotrophic factor in the mouse brain exacerbated neuronal damage and mutant androgen receptor *** findings suggest that inhibition of mesencephalic astrocyte-derived neurotrophic factor expression by mutant androgen receptor is a potential mechanism underlying neurodegeneration in spinal and bulbar muscular atrophy.

关键词： androgen receptor mesencephalic astrocyte-derived neurotrophic factor mouse model neurodegeneration neuronal loss neurotrophic factor polyglutamine disease protein misfolding spinal and bulbar muscular atrophy transcription factor

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Rock fracture mechanism of buffer blasting with cushion layer at the borehole bottom

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International Journal of Minerals,Metallurgy and Materials 2025年第2期32卷 325-334页

作者： Xinguang Zhu Chenxi Ding Zhe sui Hong su Xu Guo School of Civil and Resources Engineering University of Science and Technology BeijingBeijing 100083China Key Laboratory for Mechanics in Fluid Solid Coupling Systems Institute of MechanicsChinese Academy of SciencesBeijing 100190China School of Mechanics and Civil Engineering China University of Mining and Technology(Beijing)Beijing 100083China School of Chemical and Blasting Engineering Anhui University of Science and TechnologyHuainan 232001China

This study primarily investigates the rock fracture mechanism of bottom cushion layer blasting and explores the effects of the bottom cushion layer on rock *** involves analyses of the evolution patterns of blasting stress,characteristics of crack distribution,and rock fracture features in the ***,blasting model experiments were carried out using the dynamic caustics principle to investigate the influence of bottom cushion layers and initiation methods on the integrity of the bottom rock *** experimental results indicate that the combined use of bottom cushion layers and inverse initiation effectively protects the integrity of the bottom rock ***,the process of stress wave propagation and dynamic crack propagation in rocks was simulated using the continuum-discontinuum element method(CDEM)and the Landau explosion source model,with varying thicknesses of bottom cushion *** numerical simulation results indicate that with increasing cushion thickness,the absorption of energy generated by the explosion becomes more pronounced,resulting in fewer cracks in the bottom rock *** illustrates the positive role of the cushion layer in protecting the integrity of the bottom rock mass.

关键词： bottom cushion layer blasting crack propagation continuum-discontinuum element method dynamic stress intensity factor

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