检索结果-内蒙古大学图书馆

SSRN 2022年

作者： Tsai, Yih-Jeng Ho, Man-Hau Yen, Chia-Hung Wang, Jia-Yi Burnouf, Thierry Chen, Chia-Yen Wang, Yun Chiang, Yung-Hsiao Hoffer, Barry J. Chou, Szu-Yi Department of Otolaryngology Head and Neck Surgery Shin Kong Wu HoSu Memorial Hospital Taipei Taiwan School of Medicine Fu Jen Catholic University New Taipei City Taiwan Ph.D. Program in Medical Neuroscience College of Medical Science and Technology Taipei Medical University National Health Research Institute Taiwan Graduate Institute of Neural Regenerative Medicine College of Medical Science and Technology Taipei Medical University Taipei Taiwan Department of Biological Science and Technology National Pingtung University of Science and Technology Neipu Pingtung91201 Taiwan Graduate Institute of Medical Sciences College of Medicine Taipei Medical University Taipei11031 Taiwan Department of Neurosurgery Taipei Medical University Hospital Taipei11031 Taiwan Neuroscience Research Center Taipei Medical University Taipei11031 Taiwan International PhD Program in Biomedical Engineering College of Biomedical Engineering Taipei Medical University Taipei Taiwan Graduate Institute of Biomedical Materials and Tissue Engineering College of Biomedical Engineering Taipei Medical University Taipei Taiwan NeuroTMULille International Laboratory Taipei Medical University Taipei Taiwan International PhD Program in Cell Therapy and Regeneration Medicine College of Medicine Taipei Medical University Taipei Taiwan Brain and Consciousness Research Centre TMU Shuang Ho Hospital New Taipei City Taiwan Center for Neuropsychiatric Research National Health Research Institutes Zhunan Miaoli County Taiwan Department of Surgery School of Medicine College of Medicine Taipei Medical University 11031 Taiwan Department of Neurosurgery Case Western Reserve University School of Medicine ClevelandOH United States Scientist Emeritus National Institutes of Health United States International Master Program in Medical Neuroscience College of Medical Science and Technology Taipei Medical University Taipei Taiwan

Cytokines and chemokines play important roles in inflammation and repair following brain injury. M1 microglia are pro-inflammatory which produce cytokines and lead to neural injury;in contrast, M2 microglia are anti-inflammatory, release neurotrophic factors, and promote neural repair processes. Chemokine CCL5, increased after TBI, shows a neuroprotective function by reducing oxidative stress. CCL5 may contribute to the balance between oxidative stress and immune responses after brain injury. Herein, we investigated the role of CCL5 in microglial polarization after mild traumatic brain injury, focusing on the cerebral cortex.C57BL/6 and CCL5 knockout (CCL5-KO) mice were given a mild traumatic brain injury (TBI) using weight-drop. Neurological parameters such as motor and sensory functions were analyzed by mNSS score, accelerating rotarod, beam walking, and adhesive removal tests. Oxidative stress and neuron damage were measured by NADPH oxidase activity, hypoxyloprobe staining and FJC. Performance of motor and sensory functions in both WT and CCL5-KO mice were impaired after brain injury which recovered after 7 days-post-injury (dpi) in the WT group but only after 14 days in the CCL5-KO mouse group. RT-qPCR analysis revealed that pro-inflammation cytokines - IL-1β, TNF-α, and IL-6 were higher in CCL5-KO mice compared to WT mice at 4 and 14 dpi. In contrast, M2-like microglia markers - IL-10 and Agr-1 were increased in WT mouse cortical tissue at 4 dpi. Oxidative stress increased both M1 and M2- related cytokine expression in BV2 cells treated with H 2 O 2;CCL5 treatment increased M2- but suppressed M1- related cytokine gene activation. Intranasal delivery of CCL5 reduced neuronal oxidative stress, increased IL-10 expression and improved motor and sensory functions in CCL5-KO mice after brain *** summary, CCL5, which alters immune responses and protects neurons from TBI damage, has an important function in regulating M2-like microglial polarization during post-

关键词： Polarization

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Changing life expectancy in European countries 1990–2021: a subanalysis of causes and risk factors from the Global Burden of Disease Study 2021

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The Lancet Public Health 2025年第3期10卷 e172-e188页

作者： Steel, Nicholas Bauer-Staeb, Clarissa Maria Mercedes Ford, John A. Abbafati, Cristiana Abdalla, Mohammed Altigani Abdelkader, Atef Abdi, Parsa Zuñiga, Roberto Ariel Abeldaño Abiodun, Olugbenga Olusola Abolhassani, Hassan Abu-Gharbieh, Eman Abukhadijah, Hana J. Abu-Zaid, Ahmed Addo, Isaac Yeboah Addolorato, Giovanni Adekanmbi, Victor Adetunji, Juliana Bunmi Adeyeoluwa, Temitayo Esther Agardh, Emilie E. Agyemang-Duah, Williams Ahmad, Danish Ahmed, Ayman Ahmed, Syed Anees Akinosoglou, Karolina Akkaif, Mohammed Ahmed Al Awaidy, Salah Al Hasan, Syed Mahfuz Al Zaabi, Omar Ali Mohammed Aldridge, Robert W. Algammal, Abdelazeem M. Al-Gheethi, Adel Ali Saeed Ali, Abid Ali, Mohammed Usman Ali, Syed Shujait Ali, Waad Alicandro, Gianfranco Alif, Sheikh Mohammad Al-Jumaily, Adel Allebeck, Peter Alrawashdeh, Ahmad Al-Rifai, Rami H. Alsabri, Mohammed A. Alshahrani, Najim Z. Aluh, Deborah Oyine Al-Wardat, Mohammad Al-Zyoud, Walid A. Amiri, Sohrab Anderlini, Deanna Andrei, Catalina Liliana Anil, Abhishek Anvari, Saeid Anyasodor, Anayochukwu Edward Appiah, Seth Christopher Yaw Aquilano, Michele Arabloo, Jalal Arafat, Mosab Areda, Demelash Aremu, Abdulfatai Armani, Keivan Armocida, Benedetta Ärnlöv, Johan Asaduzzaman, Muhammad Astell-Burt, Thomas Aujayeb, Avinash Ausloos, Marcel Azadnajafabad, Sina Aziz, Shahkaar Azzam, Ahmed Y. Babu, Giridhara Rathnaiah Badache, Andreea Corina Badiye, Ashish D. Bahramian, Saeed Baig, Atif Amin Baker, Jennifer L. Bansal, Hansi Bärnighausen, Till Winfried Barone, Mark Thomaz Ugliara Barrow, Amadou Barteit, Sandra Bashir, Shahid Bashiru, Hameed Akande Basso, João Diogo Bastan, Mohammad-Mahdi Basu, Sanjay Batra, Kavita Bauckneht, Matteo Baune, Bernhard T. Beghi, Massimiliano Beiranvand, Maryam Béjot, Yannick Bell, Michelle L. Bello, Olorunjuwon Omolaja Belo, Luis Beloukas, Apostolos Beneke, Alice A. Bettencourt, Paulo J.G. Bhagavathula, Akshaya Srikanth Bhala, Neeraj Bhaskar, Sonu Bisulli, Francesca Bjørge, Tone Bodunrin, Aadam Olalekan Carvajal, Alejandro Botero Bouaoud, Souad Brayne, Carol Brenner, H Department of Primary Care and Public Health University of East Anglia Norwich United Kingdom Office for Health Improvement and Disparities Department of Health and Social Care London United Kingdom Department of Legal and Economic Studies La Sapienza University Rome Italy Rome Italy Hull York Medical School University of Hull Hull United Kingdom Department of Mathematics and Sciences Ajman University Ajman United Arab Emirates Department of Medicine Memorial University St. John's NL Canada Postgraduate Department University of Sierra Sur Miahuatlan de Porfirio Diaz Mexico Yhteiskuntadatatieteen keskus (Centre for Social Data Science) Helsinki Finland Department of Internal Medicine Federal Medical Centre Abuja Nigeria Research Center for Immunodeficiencies Tehran University of Medical Sciences Tehran Iran Department of Medical Biochemistry and Biophysics Karolinska Institutet (Karolinska Institute) Stockholm Sweden Department of Clinical Sciences University of Sharjah Sharjah United Arab Emirates Department of Biopharmaceutics and Clinical Pharmacy University of Jordan Amman Jordan Medical Research Center Hamad Medical Corporation Doha Qatar Department of Biochemistry and Molecular Medicine Alfaisal University Riyadh Saudi Arabia College of Graduate Health Sciences University of Tennessee Memphis TN United States School of Medicine Sydney NSW Australia Centre for Social Research in Health University of New South Wales Sydney NSW Australia Internal Medicine and Alcohol Related Disease Unit Fondazione Policlinico Universitario A. Gemelli IRCCS Rome Italy Department of Medical and Surgical Sciences Università Cattolica di Roma (Catholic University of Rome) Rome Italy Department of Obstetrics and Gynecology University of Texas Medical Branch Galveston TX United States Department of Biochemistry Osun State University Osogbo Nigeria Department of Pharmacology and Therapeutics University of Medical Sciences Ondo Ondo Nigeria Department of Veteri

Background: Decades of steady improvements in life expectancy in Europe slowed down from around 2011, well before the COVID-19 pandemic, for reasons which remain disputed. We aimed to assess how changes in risk factors and cause-specific death rates in different European countries related to changes in life expectancy in those countries before and during the COVID-19 pandemic. Methods: We used data and methods from the Global Burden of Diseases, Injuries, and Risk Factors Study 2021 to compare changes in life expectancy at birth, causes of death, and population exposure to risk factors in 16 European Economic Area countries (Austria, Belgium, Denmark, Finland, France, Germany, Greece, Iceland, Ireland, Italy, Luxembourg, the Netherlands, Norway, Portugal, Spain, and Sweden) and the four UK nations (England, Northern Ireland, Scotland, and Wales) for three time periods: 1990–2011, 2011–19, and 2019–21. Changes in life expectancy and causes of death were estimated with an established life expectancy cause-specific decomposition method, and compared with summary exposure values of risk factors for the major causes of death influencing life expectancy. Findings: All countries showed mean annual improvements in life expectancy in both 1990–2011 (overall mean 0·23 years [95% uncertainty interval [UI] 0·23 to 0·24]) and 2011–19 (overall mean 0·15 years [0·13 to 0·16]). The rate of improvement was lower in 2011–19 than in 1990–2011 in all countries except for Norway, where the mean annual increase in life expectancy rose from 0·21 years (95% UI 0·20 to 0·22) in 1990–2011 to 0·23 years (0·21 to 0·26) in 2011–19 (difference of 0·03 years). In other countries, the difference in mean annual improvement between these periods ranged from –0·01 years in Iceland (0·19 years [95% UI 0·16 to 0·21] vs 0·18 years [0·09 to 0·26]), to –0·18 years in England (0·25 years [0·24 to 0·25] vs 0·07 years [0·06 to 0·08]). In 2019–21, there was an overall decrease in mean annual life expectancy a

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The Diverse Genetic Landscape of Neurodevelopmental Disorders

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Genomics and Human Genetics 1000年第1期15卷 195-213页

作者： Wen F. Hu Maria H. Chahrour Christopher A. Walsh 3Harvard-MIT MD-PhD MSTP Program 2Program in Neuroscience 1Division of Genetics and Genomics Department of Medicine Manton Center for Orphan Disease Research and Howard Hughes Medical Institute Boston Children's Hospital Boston Massachusetts 02115 email: wenfanhu@hms.harvard.edu maria.chahrour@childrens.harvard.edu christopher.walsh@childrens.harvard.edu 6Autism Consortium Boston Massachusetts 02115 4Department of Pediatrics 5Department of Neurology Harvard Medical School Boston Massachusetts 02115

Advances in genetic tools and sequencing technology in the past few years have vastly expanded our understanding of the genetics of neurodevelopmental disorders. Recent high-throughput sequencing analyses of structural brain malformations, cognitive and neuropsychiatric disorders, and localized cortical dysplasias have uncovered a diverse genetic landscape beyond classic Mendelian patterns of inheritance. The underlying genetic causes of neurodevelopmental disorders implicate numerous cell biological pathways critical for normal brain development.

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Fabrication of hyaluronic acid with graphene quantum dot as a dual drug delivery system for cancer therapy

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FlatChem 2024年 44卷

作者： Lin, Joseph Lin, Jung-Hua Yeh, Tseng-Yu Zheng, Jia-Huei Cho, Er-Chieh Lee, Kuen-Chan Department of Science Education National Taipei University of Education No.134 Sec. 2 Heping E. Rd. Da-an District Taipei City 106 Taiwan School of Pharmacy College of Pharmacy Taipei Medical University 250 Wuxing Street Taipei City 110 Taiwan Master Program in Clinical Genomics and Proteomics College of Pharmacy Taipei Medical University 250 Wuxing Street Taipei City 110 Taiwan Cancer Center Wan Fang Hospital Taipei Medical University 110 Taiwan PhD Program in Medical Neuroscience College of Medical Science and Technology Taipei Medical University Taipei City 110 Taiwan

With recent advancements in nanomedicine, there has been growing interest in developing drug delivery systems with multifunctional capabilities. In this study, we developed a novel dual drug delivery system by combining two drug carriers via positive and negative electrostatic interactions. First, we modified polyethyleneimine (PEI) with graphene quantum dots (GQDs) to create a positively charged particle (GPI) with high drug loading efficiency and good dispersibility. Second, we embedded the pyrenebutyric acid structure in hyaluronic acid (HA) through EDC/NHS cross-linking and used TAK-632 as the hydrophobic drug to create negatively charged particles (HANPs) with hydrogel-like properties and the CD44 receptor. After the two components were constructed, the increase in the particle charge and simultaneous delivery of both drugs synergistically enhanced the therapeutic effect of this strategy. Qualitative tests confirmed the successful synthesis of the drug carrier, while the potential of this system as a cancer treatment strategy was evaluated in HCT116 cancer cells (through MTT cell viability assays) and in vivo mice xenograft experiments. Our results demonstrated that the dual drug delivery system, HANPs(TAK)/GPI(DOX), had a significant inhibitory effect on the growth of cancer cells both in vitro and in vivo, suggesting that this system is a promising candidate for a new type of treatment. © 2024 Elsevier B.V.

关键词： Dual drug delivery system Electrostatic interaction Graphene quantum dots Hydrophobic drug

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CuO NWs boosted triboelectric microfluidic nanosensor functionalized by collagen-protein interactions for real-time platelet count monitoring

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Chemical Engineering Journal 2024年 490卷

作者： Lin, Jia-Cheng Kaswan, Kuldeep Chatterjee, Subhodeep Wu, Yu-Wen Kumar Sharma, Manish Ranjan, Ashok Roy Barman, Snigdha Lin, Yu-Zih Burnouf, Thierry Boireau, Wilfrid Lu, Ming-Yen Tu, Yong-Kwang Su, I-Chang Wu, Ping-Hsiu Lin, Zong-Hong Fan, Yu-Jui School of Biomedical Engineering Taipei Medical University Taipei11031 Taiwan Taipei Neuroscience Institute Taipei Medical University Taipei11031 Taiwan Department of Biomedical Engineering National Taiwan University Taipei11031 Taiwan International Intercollegiate PhD Program National Tsing Hua University Hsinchu30013 Taiwan Department of Power Mechanical Engineering National Tsing Hua University Hsinchu30013 Taiwan Graduate Institute of Biomedical Materials and Tissue Engineering Taipei Medical University Taipei11031 Taiwan Department of Material Science and Engineering National Tsing Hua University Hsinchu30013 Taiwan Institute of Biomedical Engineering National Tsing Hua University Hsinchu30013 Taiwan International PhD Program in Biomedical Engineering Taipei Medical University Taipei11031 Taiwan Université de Franche-Comté CNRS Institut FEMTO-ST BesançonF-25030 France Department of Neurosurgery Shuang Ho Hospital Taipei Medical University New Taipei City23561 Taiwan Department of Surgery School of Medicine College of Medicine Taipei Medical University Taipei11031 Taiwan Department of Radiology School of Medicine College of Medicine Taipei Medical University Taipei11031 Taiwan Department of Radiation Oncology Taipei Medical University Hospital Taipei11031 Taiwan Center for Precision Health and Quantitative Sciences Taipei Medical University Hospital Taipei 11031 Taiwan

Blood platelet count significantly affects the development of severe conditions like myocardial infarction, peripheral arterial ischemia, respiratory compromise, stroke, diabetes, coronavirus disease 2019 (COVID-19), along with chemotherapy patients and those suffering cardiovascular diseases (CVDs). These conditions necessitate frequent monitoring of platelet counts to guide diagnostic and therapeutic decisions. However, existing techniques are relatively time-consuming, lack of accuracy and require precise operation. The emergence of these severe diseases underscored the need to develop advanced platelet count-monitoring techniques which are rapid, highly precise, and conveniently portable for point-of-care applications. In this study, we emphasized the development of a triboelectric microfluidic nanosensor (TMNS) for platelet quantification through the assessment of flow resistance. The functionality of TMNS device is based on immobilization of platelets on a collagen layer coated inside a microfluidic channel. The triboelectric voltage output is measured as a detection signal of the flow resistance and is enhanced by incorporating high surface area copper oxide nanowires (CuO NWs) on the interior of copper tubes. These copper tubes serve as terminal electrodes and for flow guiding. The flow resistance of plasma solutions is elevated when the platelet concentration increases due to heightened adherence of platelets onto the collagen layer. Variations in flow resistance induce alterations in contact electrification, causing changes in output voltage at load terminals. Fine-tuning of the TMNS device was achieved by optimizing the channel width and length, flowing liquid viscosity, and voltage measurement technique. Platelet quantification sensing data were acquired through the combination of platelet-rich plasma (PRP) and platelet-poor plasma (PPP) solutions. The described device exhibits promising capabilities for platelet-count monitoring in whole-blood samples c

关键词： Copper oxides

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Reliable change indices for the Italian Edinburgh Cognitive and Behavioral ALS Screen (ECAS)

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Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders 2023年第3-4期24卷 339-342页

作者： Edoardo Nicolò Aiello Federica Solca Silvia Torre Laura Carelli Alessia Monti Roberta Ferrucci Federico Verde Nicola Ticozzi Vincenzo Silani Barbara Poletti 1 IRCCS Istituto Auxologico Italiano Department of Neurology and Laboratory of Neuroscience Milano Italy2 PhD Program in Neuroscience School of Medicine and Surgery University of Milano-Bicocca Monza Italy 1 IRCCS Istituto Auxologico Italiano Department of Neurology and Laboratory of Neuroscience Milano Italy 3 Department of Neurorehabilitation Sciences Casa di Cura del Policlinico Milano Italy 4 Aldo Ravelli Center for Neurotechnology and Experimental Brain Therapeutics Department of Health Sciences International Medical School University of Milan Milano Italy5 ASST Santi Paolo e Carlo San Paolo University Hospital Milano Italy6 IRCCS Ca’ Granda Foundation Maggiore Policlinico Hospital Milano Italy 1 IRCCS Istituto Auxologico Italiano Department of Neurology and Laboratory of Neuroscience Milano Italy7 Department of Pathophysiology and Transplantation “Dino Ferrari Center” Università Degli Studi di Milano Milano Italy

This study aimed at providing standardized regression-based (SRB) reliable change indices (RCIs) for the Italian Edinburgh Cognitive and Behavioral ALS Screen (ECAS). Thirty-one consecutive ALS patients undergoing the ECAS were followed-up (T1) at 6.5 ± 1 months (range= 5–8). Ceiling/floor effects, practice effect, and test–retest reliability were assessed. Each ECAS measure was regressed by stepwise-entering as predictors demographics, respective T0 scores, T0 disease duration and ALSFRS-R, retest interval, and progression rate (ΔFS) – i.e., (48 - ALSFRS-RT0)/disease durationT0in months. Ceiling effects were infrequently detected, no practice effect emerged and all ECAS measures were reliable at retest (except for Language and Visuo-spatial subscales). T0 scores predicted all ECAS measures except for the Visuo-spatial subscale. The availability of RCIs for the Italian ECAS will aid ALS-related clinical practice and research within the longitudinal dimension.

关键词： Reliable change index Edinburgh Cognitive and Behavioral ALS Screen amyotrophic lateral sclerosis neuropsychology psychometrics

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Ludwigia octovalvis (Jacq.) P.H. Raven extract improves memory performance in mice with chronic kidney disease

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Phytomedicine Plus 2024年第2期4卷 100556-100556页

作者： Wang, Hao-Wei Huang, Yi-Hsuan Tung, Hsiao-Jou Lin, Wei-Sheng Chiu, Hao-Chieh Lin, Shuei-Liong Fan, Shou-Zen Wang, Pei-Yu Hsieh Yu Enterprise Co. Ltd. Taipei Taiwan Graduate Institute of Brain and Mind Sciences College of Medicine National Taiwan University Taipei Taiwan Department of Pediatrics Taipei Veterans General Hospital Taipei Taiwan Department of Clinical Laboratory Sciences and Medical Biotechnology College of Medicine National Taiwan University Taipei Taiwan Graduate Institute of Physiology College of Medicine National Taiwan University Taipei Taiwan Department of Anesthesiology National Taiwan University Hospital National Taiwan University Taipei Taiwan Department of Anesthesiology En Chu Kong Hospital Taipei Taiwan Neurobiology and Cognitive Science Center National Taiwan University Taipei Taiwan PhD. Program in Translational Medicine National Taiwan University and Academia Sinica Taipei Taiwan PhD. Program in Medical Neuroscience College of Medical Science and Technology Taipei Medical University Taipei Taiwan

Background: Chronic kidney disease (CKD) is a significant global health concern often accompanied by cognitive impairment in affected patients. Ludwigia octovalvis extract (LOE) is a traditional medicine widely used for various conditions, including renal disease and its associated complications. However, the pharmacological effects and underlying mechanisms of LOE on CKD remain unclear. Methods: Adult male mice underwent a 4-week oral adenine-induced CKD model, followed by daily administration of either a vehicle, losartan, or LOE for 1 to 2 months. A battery of behavioral tests was conducted to examine CKD-associated cognitive impairments. Additionally, RNA sequencing, Golgi staining, and immunohistochemistry were performed to decipher the underlying mechanisms. Results: Neither losartan nor LOE supplementation could reverse adenine-induced kidney damage. However, during behavioral tests, both losartan and LOE treatments improved memory performance in mice without affecting emotional status and general locomotor activity. Transcriptome profiling and histological analyses revealed altered dendritic arborization and increased spine density of dentate granule cells, along with augmented adult hippocampal neurogenesis, contributing to the improved memory performance of mice treated with LOE. Conclusion: Our study provides comprehensive neurobehavioral and molecular evidence supporting the potential use of LOE for future CKD-associated memory dysfunction treatment. © 2024 The Author(s)

关键词： Adenine Cognitive dysfunction Neurogenesis

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MiR34 contributes to spinal muscular atrophy and AAV9-mediated delivery of MiR34a ameliorates the motor deficits in SMA mice

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Molecular therapy. Nucleic acids 2023年 32卷 144-160页

作者： Tai-Heng Chen Shih-Hsin Chang Yu-Fu Wu Ya-Ping Yen Fang-Yu Hsu Yen-Chung Chen Yang Ming Ho-Chiang Hsu Yi-Ching Su Sheng-Tang Wong Jui-Hung Hung Shih-Hwa Chiou Yuh-Jyh Jong Jun-An Chen PhD Program in Translational Medicine Kaohsiung Medical University Kaohsiung 80708 and Academia Sinica Taipei 11529 Taiwan. Institute of Molecular Biology Academia Sinica Taipei 11529 Taiwan. Department of Pediatrics Kaohsiung Medical University Kaohsiung 80708 Taiwan. School of Post-Baccalaureate Medicine Kaohsiung Medical University Kaohsiung 80708 Taiwan. Neuroscience Program of Academia Sinica Academia Sinica Taipei 11529 Taiwan. Department of Computer Science National Yang Ming Chiao Tung University Hsinchu 30093 Taiwan. Institute of Pharmacology School of Medicine National Yang Ming Chiao Tung University Taipei 11221 Taiwan. Innovative Cellular Therapy Center Department of Medical Research Taipei Veterans General Hospital Taipei 11217 Taiwan. Department of Laboratory Medicine Kaohsiung Medical University Kaohsiung 80708 Taiwan. Translational Research Center of Neuromuscular Diseases Kaohsiung Medical University Hospital Kaohsiung Medical University Kaohsiung 80708 Taiwan. Graduate Institute of Clinical Medicine College of Medicine Kaohsiung Medical University Kaohsiung 80708 Taiwan. Drug Development and Value Creation Research Center Kaohsiung Medical University Kaohsiung 80708 Taiwan. Department of Biological Science and Technology National Yang Ming Chiao Tung University Hsinchu 30093 Taiwan.

Spinal muscular atrophy (SMA) is a neurodegenerative disease characterized by the selective loss of spinal motor neurons (MNs) and concomitant muscle weakness. Mutation of is known to cause SMA, and restoring SMN protein levels via antisense oligonucleotide treatment is effective for ameliorating symptoms. However, this approach is hindered by exorbitant costs, invasive procedures, and poor treatment responses of some patients. Here, we seek to circumvent these hurdles by identifying reliable biomarkers that could predict treatment efficacy. We uncovered that MiR34 exhibits consistent downregulation during SMA progression in both human and rodent contexts. Importantly, family-knockout mice display axon swelling and reduced neuromuscular junction (NMJ) endplates, recapitulating SMA pathology. Introducing MiR34a via scAAV9 improved the motor ability of SMNΔ7 mice, possibly by restoring NMJ endplate size. Finally, we observed a consistent decreasing trend in MiR34 family expression in the cerebrospinal fluid (CSF) of type I SMA patients during the loading phase of nusinersen treatment. Baseline CSF MiR34 levels before nusinersen injection proved predictive of patient motor skills 1 year later. Thus, we propose that MiR34 may serve as a biomarker of SMA since it is associated with the pathology and can help evaluate the therapeutic effects of nusinersen.

关键词： ASO MT: Non-coding RNAs MiR34 SMA biomarker microRNA nusinersen spinal muscular atrophy

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Comparison of endoscopic third ventriculostomy versus cerebrospinal fluid shunt procedures for the treatment of pediatric hydrocephalus in Taiwan

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Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 2024年第9期40卷 2883-2891页

作者： Shu-Mei Chen Li-Ying Chen Jiann-Her Lin Nicole Salazar Tu-Hsueh Yeh Wei-Lun Lo Tai-Ngar Lui Yi-Chen Hsieh Li-Nien Chien Department of Neurosurgery Taipei Medical University Hospital Taipei Medical University Taipei Taiwan. Department of Surgery School of Medicine Taipei Medical University Taipei Taiwan. Health Data Analytics and Statistics Center Office of Data Science Taipei Medical University Taipei Taiwan. Julius L. Chambers Biomedical/Biotechnology Institute and Department of Biological & Biomedical Sciences North Carolina Central University Durham NC USA. Department of Neurology Taipei Medical University Hospital Taipei Taipei Medical University Taipei Taiwan. Department of Neurology School of Medicine College of Medicine Taipei Medical University Taipei Taiwan. Department of Neurosurgery Shuang Ho Hospital Taipei Medical University Taipei Taiwan. The PhD Program in Medical Neuroscience College of Medical Science and Technology Taipei Medical University 250 Wu-Hsing Street Taipei Taiwan. ychsieh@tmu.edu.tw. Institute of Health and Welfare Policy College of Medicine National Yang Ming Chiao Tung University No.155 Sec. 2 Linong St. Beitou District Taipei Taiwan. linien.chien@nycu.edu.tw. Graduate Institute of Data Science College of Management Taipei Medical University Taipei Taiwan. linien.chien@nycu.edu.tw.

PURPOSE:Pediatric hydrocephalus is the most common cause of surgically treatable neurological disease in children. Controversies exist whether endoscopic third ventriculostomy (ETV) or cerebrospinal fluid (CSF) shunt placement is the most appropriate treatment for pediatric hydrocephalus. This study aimed to compare the risk of re-operation and death between the two procedures. METHODS:We performed a retrospective population-based cohort study and included patients younger than 20-years-old who underwent CSF shunt or ETV for hydrocephalus from the Taiwan National Health Insurance Research Database. RESULTS:A total of 3,555 pediatric patients from 2004 to 2017 were selected, including 2,340 (65.8%) patients that received CSF shunt placement and 1215 (34.2%) patients that underwent ETV. The incidence of all-cause death was 3.31 per 100 person-year for CSF shunt group and 2.52 per 100 person-year for ETV group, with an adjusted hazard ratio (HR) of 0.79 (95% confidence interval [CI] = 0.66-0.94, p = 0.009). The cumulative incidence competing risk for reoperation was 31.2% for the CSF shunt group and 26.4% for the ETV group, with an adjusted subdistribution HR of 0.82 (95% CI = 0.70-0.96, p = 0.015). Subgroup analysis showed that ETV was beneficial for hydrocephalus coexisting with brain or spinal tumor, central nervous system infection, and intracranial hemorrhage. CONCLUSION:Our data indicates ETV is a better operative procedure for pediatric hydrocephalus when advanced surgical techniques and instruments are available.

关键词： Cerebrospinal shunt Pediatric hydrocephalus Reoperation Third ventriculostomy

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Correction to: Metachromatic leukodystrophy with late adult-onset: diagnostic clues and differences from other genetic leukoencephalopathies with dementia

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Journal of neurology 2021年第5期268卷 1977-1979页

作者： Chiara Benzoni Marco Moscatelli Silvia Fenu Anna Venerando Ettore Salsano Unit of Rare Neurodegenerative and Neurometabolic Diseases Fondazione IRCCS Istituto Neurologico Carlo Besta Via Celoria 11 Milan 20133 Italy. chiara.benzoni1@unimi.it. Unit of Neuroradiology Fondazione IRCCS Istituto Neurologico Carlo Besta Milan Italy. Unit of Rare Neurodegenerative and Neurometabolic Diseases Fondazione IRCCS Istituto Neurologico Carlo Besta Via Celoria 11 Milan 20133 Italy. Unit of Medical Genetics and Neurogenetics Fondazione IRCCS Istituto Neurologico Carlo Besta Milan Italy. Neuroscience PhD Program University of Milano-Bicocca Monza Italy.

The original version of this article unfortunately contained a mistake.

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