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Progress in treatment and newborn screening for Duchenne muscular dystrophy and spinal muscular atrophy

World Journal of Pediatrics 2019年第3期15卷 219-225页

作者： Qing Ke Zheng-Yan Zhao Jerry R.mendell mei Baker Veronica Wiley Jennifer m.Kwon Lindsay N.Alfano anne m.connolly Catherine Jay Hanna Polari Emma Ciafaloni ming Qi Robert C.Griggs michele A.Gatheridge Department of Neurology The First Affiliated Hospital College of MedicineZhejiang UniversityHangzhouZhejiangChina Children’S Hospital Zhejiang University School of Medicine HangzhouChina Department of Pediatrics and Neurology Nationwide Children’S HospitalColumbusOHUSA Department of Pediatrics University of Wisconsin School of Medicine and Public HealthMadisonWIUSA Disciplines of Genetic medicine and Pediatric and Child Health University of SydneySydneyAustralia Department of Neurology University of Wisconsin School of Medicine and Public HealthMadisonWIUSA Department of Pediatrics Nationwide Children’S HospitalColumbusOHUSA Department of Neurology Washington University School of MedicineSt.LouisMOUSA Department of Neurology University of Rochester School of Medicine and DentistryRochesterNY’USA PerkinElmer Inc TurkuFinland Department of Clinical Laboratory Zhejiang University School of MedicineHangzhouChina Department of Neurology University of Rochester School of Medicine and Dentistry60 1 Elmwood AveBox 673RochesterNY 14642USA

Background Advances in treatment for Duchenne muscular dystrophy (DmD) and spinal muscular atrophy (SmA) hold promise for children with these *** genetic diagnosis,early in the disease process,will allow these treatments to be most *** screening (NBS) for SmA has been recommended in the United States,and a pilot DmD NBS program is underway in Hangzhou,*** sources A Pubmed search,limited to the past 5 years,was conducted to identify:(1) therapeutic advancements for DmD/SmA approved by the United States Food and Drug Administration or the European medicine Agency and (2) The status of NBS for DmD/*** We review the current state of approved treatments for DmD/*** present recommendations regarding the future of NBS for these diseases,with a focus on the outcomes and challenges of SmA NBS in New York,USA,and the DmD NBS pilot program in Hangzhou,*** Approved treatments for DmD and SmA may change the natural history of these ***-term studies of these treatments are *** avoid the known diagnostic delay associated with these disorders and provide optimal effectiveness of these treatments,early identification of patients through NBS will be *** comprehensive follow-up plans for positively identified patients will need to be in place for NBS programs to be successful.

关键词： Duchene muscular dystrophy Spinal muscular atrophy Newborn screening Neuromuscular disorders Neurology

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Self‑Healing Dynamic Hydrogel microparticles with Structural Color for Wound management

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Nano-micro Letters 2024年第11期16卷 177-190页

作者： Li Wang Xiaoya Ding Lu Fan anne m.Filppula Qinyu Li Hongbo Zhang Yuanjin Zhao Luoran Shang Department of General Surgery Ruijin HospitalShanghai Jiaotong University School of MedicineShanghai 200025People’s Republic of China Department of Rheumatology and Immunology Nanjing Drum Tower HospitalSchool of Biological Science and Medical EngineeringSoutheast UniversityNanjing 210096People’s Republic of China Pharmaceutical Sciences Laboratory Åbo Akademi University20520 TurkuFinland Oujiang Laboratory(Zhejiang Lab for Regenerative medicine Vision and Brain Health)Wenzhou InstituteUniversity of Chinese Academy of SciencesWenzhou 325001People’s Republic of China Shanghai Xuhui Central Hospital Zhongshan‑Xuhui Hospitaland the Shanghai Key Laboratory of Medical Epigeneticsthe International Co‑laboratory of Medical Epigenetics and Metabolism(Ministry of Science and Technology)Institutes of Biomedical SciencesFudan UniversityShanghai 200032People’s Republic of China

Chronic diabetic wounds confront a significant medical challenge because of increasing prevalence and difficult-healing *** is vital to develop multifunctional hydrogel dressings,with well-designed morphology and structure to enhance flexibility and effectiveness in wound *** achieve these,we propose a self-healing hydrogel dressing based on structural color microspheres for wound *** microsphere comprised a photothermal-responsive inverse opal framework,which was constructed by hyaluronic acid methacryloyl,silk fibroin methacryloyl and black phosphorus quantum dots(BPQDs),and was further re-filled with a dynamic *** dynamic hydrogel filler was formed by Knoevenagel condensation reaction between cyanoacetate and benzaldehyde-functionalized dextran(DEX-CA and DEX-BA).Notably,the composite microspheres can be applied arbitrarily,and they can adhere together upon near-infrared irradiation by leveraging the BPQDs-mediated photothermal effect and the thermoreversible stiffness change of dynamic ***,eumenitin and vascular endothelial growth factor were co-loaded in the microspheres and their release behavior can be regulated by the same ***,effective monitoring of the drug release process can be achieved through visual color *** microsphere system has demonstrated desired capabilities of controllable drug release and efficient wound *** characteristics suggest broad prospects for the proposed composite microspheres in clinical applications.

关键词： Black phosphorus Structural color Dynamic hydrogel Inverse opal Wound management

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Assessing gully erosion and rehabilitation using multi temporal LiDAR DEms:Case study from the Great Barrier Reef catchments,Australia

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International Soil and Water Conservation Research 2024年第1期12卷 184-199页

作者： Sana Khan Rebecca Bartley anne Kinsey-Henderson Aaron Hawdon The Commonwealth Scientific and Industrial Research Organisation(CSIRO) Environment1 James Cook DrDouglas4814Australia The Commonwealth Scientific and Industrial Research Organisation(CSIRO) EnvironmentGPO Box 2583BrisbaneQLD4001Australia

millions of dollars are being spent on gully rehabilitation to help reduce excess fine sediment delivery to the Great Barrier Reef(GBR).There is an urgent need for(i)prioritisation of active gullies for rehabilitation and(ii)the development of methodologies to inform the effectiveness of *** this study we analyse DEms of Difference derived from 0.5 m resolution 2-3 year interval multi-temporal LiDAR data collected pre and post rehabilitation at three variable gully morphologies in the Burdekin *** analysis indicates that the highest annual average fine sediment erosion rates for the untreated control gullies occur at the linear gully(53.38 t ha^(-1)y^(-1))followed by linear-alluvial gully(34.24 t ha^(-1)y^(-1))and least at the alluvial gully(14.41 t ha^(-1)y^(-1)).The proportional loss or export of fine sediment from the gullies in their un-treated condition ranges from∼68 to 90%of what is eroded,and when the gullies are treated the proportion of fine sediment that is retained in the gully proportional to what is eroded increases to∼60%at all *** pre-treatment baseline erosion rates,and additional post treatment LiDAR captures,it is difficult to quantify the treatment *** results offer insights in the erosion mechanisms within different geomorphic gully morphologies and rehabilitation effects in these erosional *** study provides crucial knowledge of gully dynamics that can be coupled with other lines of evidence for better prioritisation of rehabilitation in the GBR catchments.

关键词： Digital elevation models (dis)connectivity Restoration Geomorphic change detection Geomorphic effectiveness GIS

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Biochar-compost-based controlled-release nitrogen fertilizer intended for an active microbial community

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Frontiers of Agricultural Science and Engineering 2024年第2期11卷 326-343页

作者： Robiul Islam RUBEL Lin WEI Salman ALANAZI Abdulkarim ALDEKHAIL anne CmCIDREIRA Xufei YANG Sanjita WASTI Samarthya BHAGIA Xianhui ZHAO Department of Agricultural and Biosystem Engineering South Dakota State UniversityBrookingsSD 57007USA Tickle College of Engineering University of Tennessee KnoxvilleKnoxvilleTN 37996USA Oak Ridge National Laboratory Oak RidgeTN 37830USA

Nitrogen(N) fertilizers in agriculture suffer losses by volatilization of N to the air, surface runoff and leaching into the soil, resulting in low N use efficiency(NUE)( 50%) and raising severe environmental pollutions. Controlledrelease nitrogen fertilizers(CRNFs) can control the release of N nutrients to NUE in crop production. Different methods were used to develop new ***, different CRNF technologies are still underdeveloped due to inadequate controlling on N releasing time and/or unsustainable diffusion. The study on the influences of CRNF processing parameters on microbial conditions are lacking when the CRNFs composed of various bio-ingredients such as biochar, composts, and biowaste. The complexity of processing methods, material biodegradability, and other physical properties make current CRNFs of questionable value in agricultural production. This research aims to develop a novel biochar-compost-based controlled-release urea fertilizer(BCRUF) to preserve microbial properties carried by the compost. The BCRUF was synthesized by pelletizing the 50:50(dry, wt/wt) mixture of biochar and compost. BCRUF was loaded with urea and then spray-coated with polylactic acid(PLA). The releasing time of two types of BCRUFs, coated and uncoated with PLA, for 80% of N release in water was up to 6 h at three different temperatures(4, 23, and 40 °C), compared to conventional urea fertilizer and commercial environmentally smart N(ESN) fertilizer. The releasing time of coated BCRUF for 80% N release in soil was up to 192 h(8 days). Fourier-transform infrared spectroscopy(FTIR) analysis revealed that no new functional groups were found in the release solution, indicating no new chemical hazards generated. The differential scanning calorimetry(DSC)tests also verified that its thermal stability could be up to 160 °C. The microbe populations in the BCRUF pellets were reduced after the pelleting and drying processes in BCRUF fabrication, but a few bacteria can endure in t

关键词： Soil microbial community biochar compost controlled-release nitrogen fertilizer polylactic acid spray coating

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Rethinking neurodegenerative diseases:neurometabolic concept linking lipid oxidation to diseases in the central nervous system

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Neural Regeneration Research 2024年第7期19卷 1437-1445页

作者： Steinunn Sara Helgudóttir anne Skøttrup mørkholt Jacek Lichota Preben Bruun-Nyzell mads Christian Andersen Nanna marie Juhl Kristensen Amanda Krøger Johansen mikela Reinholdt Zinn Hulda maria Jensdóttir John Dirk Vestergaard Nieland 2N Pharma ApS NOVI Science ParkAalborgDenmark molecular Pharmacology Group Department of Health Science and TechnologyAalborg UniversityAalborgDenmark

Currently,there is a lack of effective medicines capable of halting or reve rsing the progression of neurodegenerative disorde rs,including amyotrophic lateral sclerosis,Parkinson s disease,multiple sclerosis,or Alzheimer s *** the unmet medical need,it is necessary to reevaluate the existing para digms of how to to rget these *** considering neurodegenerative diseases from a systemic neurometabolic perspective,it becomes possible to explain the shared pathological *** innovative approach presented in this paper draws upon exte nsive research conducted by the authors and researchers *** this review,we highlight the importance of metabolic mitochondrial dysfunction in the context of neurodegenerative *** provide an overview of the risk factors associated with developing neurodegenerative disorders,including genetic,epigenetic,and environmental fa ***,we examine pathological mechanisms implicated in these diseases such as oxidative stress,accumulation of misfolded proteins,inflammation,demyelination,death of neurons,insulin resistance,dysbiosis,and neurotransmitter ***,we outline a proposal for the restoration of mitochondrial metabolism,a crucial aspect that may hold the key to facilitating curative therapeutic interventions for neurodegenerative disorders in forthcoming advancements.

关键词： brain disease carnitine palmitoyl transferase 1 epigenetics metabolism gut microbiome mitochondrial dysfunction neurodegeneration oxidative stress

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The dorsal root ganglion as a target for neurorestoration in neuropathic pain

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Neural Regeneration Research 2024年第2期19卷 296-301页

作者： Guillermo Estivill-Torrús Ana Belen martínez-Padilla Lourdes Sánchez-Salido anne Baron-Van Evercooren Beatriz García-Díaz Instituto de Investigación Biomédica de málaga y Plataforma en Nanomedicina(IBImA Plataforma BIONAND) MálagaSpain Unidad Clínica de Neurociencias Hospital Regional Universitario de MálagaMálagaSpain Departamento de Biología molecular y Bioquímica Universidad de MálagaSpain Sorbonne Université Institut du Cerveau-Paris Brain Institute-ICMINSERMCNRSAP-HPHôpital Pitié-SalpêtrièreParisFrance

Neuropathic pain is a severe and chronic condition widely found in the general *** reason for this is the extensive variety of damage or diseases that can spark this unpleasant constant feeling in *** the processing of pain,the dorsal root ganglia constitute an important region where dorsal root ganglion neurons play a crucial role in the transmission and propagation of sensory electrical ***,the dorsal root ganglia have recently exhibited a regenerative capacity that should not be neglected in the understanding of the development and resolution of neuropathic pain and in the elucidation of innovative ***,we will review the complex interplay between cells(satellite glial cells and inflammatory cells)and factors(cytokines,neurotrophic factors and genetic factors)that takes place within the dorsal root ganglia and accounts for the generation of the aberrant excitation of primary sensory neurons occurring in neuropathic *** importantly,we will summarize an updated view of the current pharmacologic and nonpharmacologic therapies targeting the dorsal root ganglia for the treatment of neuropathic pain.

关键词： cytokines dorsal root ganglia genetic factors neuropathic pain neurotrophic factors pharmacologic and nonpharmacologic therapies satellite glial cells sensory neurons

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The germline genetics of mild-to-moderate penetrance:An intriguing role of PRAmE in multiple carcinogenesis

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Genes & Diseases 2024年第3期11卷 7-10页

作者： Diaddin Hamdan Van Tai Nguyen Justine Paris Christophe Leboeuf morad El Bouchtaoui marc Espie anne Janin Geraldine Falgarone melanie Di Benedetto Guithem Bousquet Universite de Paris Cite INSERMUMR_S942MASCOTParis F-75006France Hopital La Porte Verte Versailles F-78004France National Cancer Hospital Cancer Research and Clinical Trials CenterHaNoi100000VietNam AP-HP-Hopital Saint Louis Centre de Maladies du SeinParisF-75010France Universite Sorbonne Paris Nord Bobigny F-93000France AP-HP-Hopital Avicenne Unite de Medecine AmbulatoireBobigny F-93000France

Germline genetics of high penetrance such as BRCA genes,mutations might be sufficient for carcinogenesis,but this only explains fewer than 10%of *** assume that most cancers are the result of germline mutations of low to moderate penetrance,combined with acquired mutations due to external carcinogenic *** their accumulation over time,aging is associated with an increased risk of multiple cancer development in a given *** decades,germline genetics have been based on familial linkage studies enabling most high-penetrance genes to be *** recently,population-wide studies have led to the identification of considerable numbers of polymorphisms associated with cancer ***,most of them are of unknown functional value,thus limiting their translational application.

关键词： carcinogenesis BRCA linkage

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Associations of accelerometry-measured and self-reported physical activity and sedentary behavior with skeletal muscle energetics:The Study of muscle,mobility and Aging(SOmmA)

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Journal of Sport and Health Science 2024年第5期13卷 621-630页

作者： Yujia(Susanna)Qiao Terri L.Blackwell Peggy m.Cawthon Paul m.Coen Steven R.Cummings Giovanna Distefano Samaneh Farsijani Daniel E.Forman Bret H.Goodpaster Stephen B.Kritchevsky Theresa mau Frederico G.S.Toledo anne B.Newman Nancy W.Glynn Department of Epidemiology School of Public HealthUniversity of PittsburghPittsburghPA 15261USA San Francisco Coordinating Center California Pacific Medical Center Research InstituteSan FranciscoCA 94143USA Department of Epidemiology and Biostatistics University of CaliforniaSan FranciscoCA 94143USA Translational Research Institute AdventHealthOrlandoFL 32804USA Department of medicine(Geriatrics and Cardiology) University of Pittsburghand GeriatricsResearchEducationand Clinical Center(GRECC)VA Pittsburgh Healthcare SystemPittsburghPA 15261USA Department of Internal medicine Section on Gerontology and Geriatric MedicineWake Forest School of MedicineWinston-SalemNC 27109USA Division of Endocrinology and metabolism Department of MedicineUniversity of PittsburghPittsburghPA 15261USA

Background:Skeletal muscle energetics decline with age,and physical activity(PA)has been shown to offset these declines in older ***,many studies reporting these effects were based on self-reported PA or structured exercise ***,we examined the associations of accelerometry-measured and self-reported PA and sedentary behavior(SB)with skeletal muscle energetics and explored the extent to which PA and sedentary behavior would attenuate the associations of age with muscle ***:As part of the Study of muscle,mobility and Aging,enrolled older adults(n=879),810(age=76.4±5.0 years old,mean±SD;58%women)had maximal muscle oxidative capacity measured ex vivo via high-resolution re spirometry of permeabilized myofibers(maximal oxidative phosphorylation(maxOXPHOS))and in vivo by ^(31)phosphorus magnetic resonance spectroscopy(maximal adenosine triphosphate(ATP_(max))).Accelerometry-measured sedentary behavior,light activity,and moderate-to-vigorous PA(mVPA)were assessed using a wrist-worn ActiGraph GT9X over 7 ***-reported sedentary behavior,mVPA,and all PA were assessed with the Community Healthy Activities model Program for Seniors(CHAmPS)*** regression models with progressive covariate adjustments evaluated the associations of sedentary behavior and PA with muscle energetics,as well as the attenuation of the age/muscle energetics association by mVP A and sedentary *** a sensitivity analysis,we also examined activPAL-measured daily step count and time spent in sedentary behavior and their associations with muscle ***:Every 30 min/day more of ActiGraph-measured mVPA was associated with 0.65 pmol/(s×mg)higher maxOXPHOS and 0.012 mm/s higher ATP_(max)after adjusting for age,site/technician,and sex(pmaxOXPHOS or ATP_(max).meanwhile,every 30 min/day spent in ActiGraph-measured sedentary behavior was associated with 0.39 pmol/s×mg lower maxOXPHOS and0.

关键词： Aging Exercise mitochondria

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Are there sex-based disparities in cataract surgery?

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International Journal of Ophthalmology(English edition) 2024年第1期17卷 137-143页

作者： matthew D.Geiger anne m.Lynch Alan G.Palestine Nathan C.Grove Karen L.Christopher Richard S.Davidson michael J.Taravella Naresh mandava Jennifer L.Patnaik Department of Ophthalmology University of Colorado School of MedicineAuroraCO 80045USA

AIm:To investigate sex-based differences in the occurrence of intra-operative and post-operative complications and associated visual outcomes following cataract ***:This was a retrospective study of patients who had phacoemulsification cataract surgery at the University of Colorado School of *** collected included the patient’s health history,ocular comorbidities,operative and post-operative complications,and the post-operative best corrected visual acuity(BCVA).The data were analyzed using univariate and multivariable logistic regression with generalized estimating equations to account for the correlation of some patients having two eyes included in the ***:A total of 11977 eyes from 7253 patients were included in the *** comorbidities differed by sex,with males having significantly higher percentages of traumatic cataracts(males 0.7%vs females 0.1%),prior ocular surgery(6.7%vs 5.5%),and mature cataracts(2.8%vs 1.9%).Conversely,females had significantly higher rates of pseudoexfoliation(2.0%vs 3.2%).In unadjusted analysis,males had higher rates of posterior capsular rupture(0.8%vs 0.4%)and vitreous loss(1.0%vs 0.6%),but this difference was not significant after adjustment for *** had a significantly increased risk of post-operative retinal detachment,but in multivariable analysis this was no longer *** were significantly less likely to undergo post-operative neodymium-doped yttrium aluminum garnet(Nd:YAG)laser capsulotomy for posterior capsule opacification(OR=0.8,95%CI=0.7-0.9,P=0.0005).The BCVA was slightly worse for males pre-operatively;but post-operatively,both sexes exhibited similar visual acuity of Snellen equivalent 20/***:The study finds that in a cohort of patients presenting for cataract surgery,sex differences exist in pre-operative comorbidities and surgical characteristics that contribute to higher rates of some complications for ***,observed surgical complicatio

关键词： cataract surgery sex-based disparity phacoemulsification outcomes

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Heterogeneity of mature oligodendrocytes in the central nervous system

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Neural Regeneration Research 2025年第5期20卷 1336-1349页

作者： Chao Weng Adam m.R.Groh moein Yaqubi Qiao-Ling Cui Jo anne Stratton G.R.Wayne moore Jack P.Antel Neuroimmunology Unit Montreal Neurological Institute-HospitalDepartment of Neurology and NeurosurgeryMcGill UniversityMontrealQCCanada Department of Neurology Renmin Hospital of Wuhan UniversityWuhanHubei ProvinceChina

mature oligodendrocytes form myelin sheaths that are crucial for the insulation of axons and efficient signal transmission in the central nervous *** evidence has challenged the classical view of the functionally static mature oligodendrocyte and revealed a gamut of dynamic functions such as the ability to modulate neuronal circuitry and provide metabolic support to *** the recognition of potential heterogeneity in mature oligodendrocyte function,a comprehensive summary of mature oligodendrocyte diversity is *** delve into early 20th-century studies by Robertson and Río-Hortega that laid the foundation for the modern identification of regional and morphological heterogeneity in mature ***,recent morphologic and functional studies call into question the long-assumed homogeneity of mature oligodendrocyte function through the identification of distinct subtypes with varying myelination ***,modern molecular investigations,employing techniques such as single cell/nucleus RNA sequencing,consistently unveil at least six mature oligodendrocyte subpopulations in the human central nervous system that are highly transcriptomically diverse and vary with central nervous system *** and disease related mature oligodendrocyte variation denotes the impact of pathological conditions such as multiple sclerosis,Alzheimer's disease,and psychiatric ***,caution is warranted when subclassifying mature oligodendrocytes because of the simplification needed to make conclusions about cell identity from temporally confined *** studies leveraging advanced techniques like spatial transcriptomics and single-cell proteomics promise a more nuanced understanding of mature oligodendrocyte *** research avenues that precisely evaluate mature oligodendrocyte heterogeneity with care to understand the mitigating influence of species,sex,central nervous system region,age,and disease,

关键词： aging central nervous system diseases electron microscopy heterogeneity immunohistochemistry myelin sheath natural history neuroglia oligodendroglia single-cell gene expression analysis

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