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Advances in the differentiation of pluripotent stem cells into vascular cells

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World Journal of Stem Cells 2024年第2期16卷 137-150页

作者： Yi-Chang Jiao Ying-Xin Wang Wen-Zhu Liu Jing-Wen Xu Yu-Ying Zhao Chuan-Zhu Yan Fu-Chen Liu Department of Neurology Qilu Hospital of Shandong UniversityJinan 250012Shandong ProvinceChina Research Institute of neuromuscular and Neurodegenerative Diseases Qilu Hospital of Shandong UniversityJinan 250012Shandong ProvinceChina Mitochondrial Medicine Laboratory Qilu Hospital(Qingdao)of Shandong UniversityQingdao 266103Shandong ProvinceChina Brain Science Research Institute Shandong UniversityJinan 250012Shandong ProvinceChina

Blood vessels constitute a closed pipe system distributed throughout the body,transporting blood from the heart to other organs and delivering metabolic waste products back to the lungs and *** in blood vessels are related to many disorders like stroke,myocardial infarction,aneurysm,and diabetes,which are important causes of death *** research for new appro-aches to disease modeling and effective treatment is needed due to the huge socio-economic burden on healthcare *** mice or rats have been widely used,applying data from animal studies to human-specific vascular physiology and pathology is *** rise of induced pluripotent stem cells(iPSCs)provides a reliable in vitro resource for disease modeling,regenerative medicine,and drug discovery because they carry all human genetic information and have the ability to directionally differentiate into any type of human *** review summarizes the latest progress from the establishment of iPSCs,the strategies for differentiating iPSCs into vascular cells,and the in vivo trans-plantation of these vascular *** also introduces the application of these technologies in disease modeling,drug screening,and regenerative ***,the application of high-tech tools,such as omics analysis and high-throughput sequencing,in this field is reviewed.

关键词： Induced pluripotent stem cell Blood vessels Vascular organoids Endothelial cells Smooth muscle cells Pericytes Tissue engineering vascular graft

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

Decreased Gait and Function in Duchenne Muscular Dystrophy

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Open Journal of Therapy and Rehabilitation 2014年第3期2卷 120-125页

作者： Cristina Cristina Iwabe-Marchese Aline Fávaro Lívia Cocato Luiz Child Neurology Rehabilitation outcome-UNICAMP Campinas Brazil neuromuscular outcome University State of Campinas-UNICAMP Campinas Brazil

Duchenne muscular dystrophy (DMD) is a genetic disorder linked to chromosome Xp21, due to absence of dystrophin production. It is clinically characterized by progressive muscle weakness, fatigue, and development of joint contractures that compromise general motor functionality, mainly the gait. Objective: To characterize the motor function and decrease gait in children with DMD using the Portuguese version of the Motor Function Measure scale (MFM-P). Methods: A review of medical records including chronological age and scores from MFM-P of children with a DMD who attended at the neuromuscular Diseases Clinic at Campinas State University (UNICAMP), Brazil was performed in this study. A total of 36 medical records of male patients with confirmed clinical diagnosis of DMD, ambulatory or not, regardless of age;excluding those with other associated diseases or other types of muscular dystrophies were selected. Data were analyzed using Kolmogorov-Smirnov and Spearman correlation statistical tests. Results: Analysis of all data collected showed that 75% of our sample had D1 scores lower than 41.02%. There was a linear relationship between the scores of D2 and D3, but no association between D2 and D1 scores was noted. D1 score was between 40% and 80% in those patients presenting D2 scores between 80% and 100%. In all cases patients with low total score presented a greater risk for loss of gait and their functionality. Conclusion: The standing posture and the postural transfers were the worst activities observed in children with DMD, with positive correlation between proximal and distal motor function. Even with high scores according MFM-P in proximal function, the children showed strong predictors for loss of gait.

关键词： Gait Muscular Dystrophy Duchenne Functionality Motor Function Measure Scale

Glial derived neurotrophic factor: a sufficient essential molecular regulator of mammalian blood-nerve barrier tight junction formation

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Neural Regeneration Research 2021年第7期16卷 1417-1418页

作者： Chaoling Dong Aarti Choudhary Eroboghene E.Ubogu neuromuscular Immunopathology Research Laboratory Division of Neuromuscular DiseaseDepartment of NeurologyUniversity of Alabama at BirminghamBirminghamALUSA

Peripheral nerves coordinate signal transduction from the periphery to the central nervous system for processing and transmission back as required for normal mammalian *** nerves and nerve roots are structurally divided into three compartments:the outermost epineurium,inner perineurium that surrounds nerve fascicles and the innermost endoneurium(Mizisin and Weerasuriya,2011).

关键词： function. barrier coordinate

Regenerative biomarkers for Duchenne muscular dystrophy

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Neural Regeneration Research 2019年第8期14卷 1317-1320页

作者： Simon Guiraud Kay E.Davies MDUK Oxford neuromuscular Centre Department of PhysiologyAnatomy and Genetics

Skeletal muscle has an extraordinary capacity to regenerate after injury and trauma. The muscle repair mechanism is a complex process orchestrated by multiple steps. In neuromuscular disorders such as Duchenne muscular dystrophy (DMD), the pathological consequences of the lack of dystrophin and the loss of the dystrophin-associated protein complex are dramatic, with a progressive cascade of events, such as continual influx of inflammation, repeated cycles of degeneration and impaired regeneration. Thus, muscle regeneration is a hallmark of the disease and careful monitoring of regenerative processes with robust markers should provide useful information to the field. Since decades, several indices of regeneration such as centronucleation and fibre size have been commonly used. In the present review, we discuss the impaired regenerative process in DMD, the common and new indices of regeneration and their associated methodologies. We notably highlight the regenerative marker embryonic myosin as a robust indicator of muscle regeneration. We also describe new quantitative methodologies offering the possibility of using a panel of translational regenerative biomarkers to obtain a more complete view of the regeneration processes. Upregulation of utrophin, an autosomal and functional paralogue of dystrophin, is one of the most promising therapeutic strategies as it targets the primary cause of the disease and is applicable to all DMD patients regardless their genetic defects. As utrophin is a regeneration associated protein increased in dystrophic muscle, we discuss the correlation of utrophin levels after drug treatment with regeneration markers. The recent advances in technologies and complementary markers of muscle regeneration described in this review, provide an unprecedented opportunity to develop more robust utrophin DMD based strategies for all DMD patients.

关键词： DMD regeneration biomarkers utrophin embryonic myosin methodologies muscle repair degeneration

Beyond vertebrates:the amphioxus as a relevant model system to explore the formation,organization,and regeneration of neuromuscular synapses

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Neural Regeneration Research 2022年第11期17卷 2425-2426页

作者： Esperanza Martínez Sylvain Marcellini Juan Pablo Henríquez neuromuscular Studies Lab (NeSt Lab) Group for the Study of Developmental Processes (GDeP) Department of Cell BiologyFaculty of Biological SciencesUniversidad de Concepción Laboratory of Development and Evolution (LADE)

The neuromuscular junction(NMJ)is the peripheral synapse controlling muscle contraction and coordinated movement in a wide variety of *** humans,the mature NMJ is the primary target of morphological disassembly and fu... 详细信息

关键词： system contraction synapse

LYVE-1 is'on stage'now:an emerging player in dendritic cell docking to lymphatic endothelial cells

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Cellular & Molecular Immunology 2018年第7期15卷 663-665页

作者： Elena Rinaldi Fulvio Baggi Neurology IV-Neuroimmunology and neuromuscular Diseases Unit Neurological Institute‘Carlo Besta’MilanItaly

The lymphatic system consists of a network of lymph capillaries and vessels deputed to drain the interstitial fluid(containing floating macromolecules,such as nutrients,cellular debris and tissue-infiltrating pathogens)and to transport tissue-patrolling lymphocytes and dendritic cells(DCs)to draining lymph nodes(drLNs).1 Knowledge of the mechanisms associated with cellular transmigration into the lymphatic system could offer the opportunity to manipulate the immune response in a wide range of diseases,such as autoimmune diseases and cancer.2,3 In this research highlight,we discuss a recent study that sheds light on our understanding of the molecules involved in DCs docking to lymphatic endothelial cells,a critical step prior to their subsequent endothelial transmigration into lymphatic *** docking occurs through the interaction between hyaluronan(hyaluronic acid,HA)molecules on the plasma membrane and lymphatic-vessel endothelial protein(LYVE-1).

关键词： lymphatic drain floating

A longitudinal analysis of the U.S. Air Force Reserve Officers’ Training Corps physical fitness assessment

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Military Medical Research 2020年第2期7卷 166-173页

作者： Cameron SMackey Jason MDe Freitas Applied neuromuscular Physiology Laboratory Oklahoma State University192 CRCStillwaterOK 74078USA

Background:The *** Force physical fitness assessment(PFA)is used to determine the overall fitness of their *** is currently unknown to what extent the PFA scores of Reserve Officers’Training Corps(ROTC)cadets are affected by mandatory physical *** purpose of this investigation was to longitudinally examine the PFAs of ROTC cadets over a four-year period,evaluate the results across class ranks,and evaluate the sensitivity of the classification of the ***:Air Force ROTC cadets performed the PFAs(abdominal circumference,1-min pushups,1-min sit-ups,and a 1.5-mile run)in both the spring(n=26)and fall(n=22)*** were compiled over a four-year period(Spring 2014–Fall 2017)and were performed in accordance with Air Force Instruction 36–2905.A oneway repeated measures ANOVA was performed separately for the fall and spring groups for each dependent variable across the 4 ***,a one-way between groups ANOVA was performed for each dependent variable during the time point(fall 2015;n=46)with the most recorded cadets for each class ***:Longitudinal assessments revealed a main effect of time(P=0.010)on abdominal circumference;cadets had a smaller abdominal circumference in their freshman year than in their senior year.A main effect of time(P=0.006)was also observed on sit-up quantity;cadets performed more sit-ups in their junior year than in their freshman *** between class ranks during the same year(between-subjects ANOVA)revealed a main effect of class rank on sit-up quantity(P=0.003);the freshmen completed fewer repetitions than the sophomores(P=0.018)and the juniors did(P=0.001).Conclusions:The results indicated that only the sit-up component showed differences between class *** findings suggest that the Air Force PFA may not be sensitive enough to detect changes in physical fitness or distinguish between class ranks regarding physical performance,even after years of *** limitation may b

关键词： Class ranks Fitness assessment Military Physical training ROTC

Tailoring of therapy for chronic inflammatory demyelinating polyneuropathy

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Neural Regeneration Research 2015年第9期10卷 1399-1400页

作者： Yusuf A.Rajabally Regional neuromuscular Clinic Queen Elizabeth Hospital University Hospitals of Birmingham

Chronic inflammatory demyelinating polyneuropathy （CIDP） is a treatable immune-mediated disorder, which causes in its typical form, symmetric proximal and distal weakness with large fibre sensory impairment involving the four limbs. There are currently three main first-line therapeutic options for CIDP. These consist of corticosteroids, immunoglobulins and plasma exchanges （PE） which have all been found effective in a number of trials conducted over the past several years （Van den Bergh and Rajabally, 2013）. No immunosuppressant therapy has shown benefit in CIDP, although they are utilized by many clinicians in various circumstances despite absence of an evidence base.

关键词： CIDP Tailoring of therapy for chronic inflammatory demyelinating polyneuropathy oral