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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

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

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

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

Small heat shock protein B8:from cell functions to its involvement in diseases and potential therapeutic applications

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Neural Regeneration research 2025年第10期20卷 2872-2886页

作者： Marta Chierichetti Riccardo Cristofani Valeria Crippa Veronica Ferrari Marta Cozzi Elena Casarotto Paola Pramaggiore Laura Cornaggia Guglielmo Patelli Ali Mohamed Margherita Piccolella Mariarita Galbiati Paola Rusmini Barbara Tedesco Angelo Poletti laboratory of Experimental Biology Dipartimento di Scienze Farmacologiche e Biomolecolari“Rodolfo Paoletti”(DiSFeB)Universitàdegli Studi di MilanoMilanItaly

Heat shock protein family B(small)member 8(HSPB8)is a 22 kDa ubiquitously expressed protein belonging to the family of small heat shock ***8 is involved in various cellular mechanisms mainly related to proteotoxic stress response and in other processes such as inflammation,cell division,and ***8 binds misfolded clients to prevent their aggregation by assisting protein refolding or degradation through chaperone-assisted selective *** line with this function,the pro-degradative activity of HSPB8 has been found protective in several neurodegenerative and neuromuscular diseases characterized by protein misfolding and *** cancer,HSPB8 has a dual role being capable of exerting either a pro-or an anti-tumoral activity depending on the pathways and factors expressed by the model of cancer under ***,HSPB8 exerts a protective function in different diseases by modulating the inflammatory response,which characterizes not only neurodegenerative diseases,but also other chronic or acute conditions affecting the nervous system,such as multiple sclerosis and intracerebellar *** note,HSPB8 modulation may represent a therapeutic approach in other neurological conditions that develop as a secondary consequence of other *** is the case of cognitive impairment related to diabetes mellitus,in which HSPB8 exerts a protective activity by assuring mitochondrial *** review aims to summarize the diverse and multiple functions of HSPB8 in different pathological conditions,focusing on the beneficial effects of its ***-based and alternative therapeutic approaches targeting HSPB8 and its regulated pathways will be discussed,emphasizing how new strategies for cell and tissue-specific delivery represent an avenue to advance in disease treatments.

关键词： aggregation autophagy cancer chaperone chaperone-assisted selective autophagy cognitive impairment HSPB8 neuroinflammation neuromuscular diseases therapy

Regional neuromuscular regulation within rectus femoris muscle following three-month limb-loaded walking in older adults

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Sports Medicine and Health Science 2022年第1期4卷 38-43页

作者： Shideh Narouei Hiroyasu Akatsu Kohei Watanabe laboratory of neuromuscular Biomechanics Faculty of Liberal Studies and Sciences and School of International Liberal StudiesChukyo UniversityNagoyaJapan Department of Community-based Medical Education Nagoya City University Graduate School of Medical ScienceNagoyaJapan

Limb-loaded walking using ankle weights has been widely applied to increase exercise intensity in older *** changes in the activation pattern between proximal(RFP)and distal(RFD)regions of the rectus femoris(RF)muscle is key to clarifying gait deficits in older *** aim of this study was to determine regional neuromuscular regulation within the RF muscle following three-month limb-loaded walking in older *** study participants were 22 healthy older adults(696.3 years)who walked at least 160 min per *** electromyography(EMG)and motion capture were used to measure the neuromuscular activities of RFP and RFD and generate kinematic data on the left lower extremity on walking for 240 s at the preferred gait speed on a treadmill at pre-and post-intervention,*** rectified values(ARV)of RFP and RFD were normalized by maximum values of ARV during a gait cycle within ten consecutive gait *** ARV of RFP was greater than RFD at 30%–40%and 70%–90%of the gait cycle and hip joint flexion at 0%–100%,and the walking speed and swing timing at post-were greater than at pre-intervention(p0.05).The activity of RFP compared with RFD and hip joint flexion were increased following limb-loaded walking intervention in older adults.

关键词： Older adults Limb-loaded walking Surface electromyography Proximal to distal region activity ratio Hip joint angle Swing phase

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

Ultra-early amplitude decrement after repetitive nerve stimulation supports early neuromuscular junction injury in amyotrophic lateral sclerosis:a prospective cross-sectional study

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Neural Regeneration research 2022年第3期17卷 655-660页

作者： Jing-Yue Ma Xiang-Yi Liu Shuo Zhang Dong-Sheng Fan Department of Neurology Peking University Third HospitalBeijingChina Beijing Municipal Key laboratory of Biomarker and Translational research in Neurodegenerative Diseases BeijingChina

The dying-back hypothesis holds that the damage to neuromuscular junctions and distal axons in amyotrophic lateral sclerosis occurs at the earliest stage of the *** basic studies have confirmed early damage to neuromuscular junctions,but it is difficult to obtain such evidence directly in clinical *** this prospective cross-sectional study,we recruited 22 patients with early amyotrophic lateral sclerosis with disease duration < 12 months and with clinical symptoms limited to the upper *** also recruited 32 healthy *** nerve stimulation was performed,and patients were followed for 12 *** found a significant change in the response to repetitive nerve stimulation in amyotrophic lateral sclerosis patients without spontaneous electromyographic *** that were prone to denervation had an increased decrement response of target muscles after repetitive nerve *** results suggest that changes in response to repetitive nerve stimulation may occur before denervation in amyotrophic lateral sclerosis *** damage to lower motor neurons is more obvious in patients with a higher percentage of repetitive never stimulation-related amplitude *** study was approved by the Institutional Ethics Committee of Peking University Third Hospital(approval No.M2017198) on August 24,2017.

关键词： amplitude decremental response amyotrophic lateral sclerosis dying-back hypothesis motor neuron disease nerve electrophysiology physiopathology prognosis repetitive nerve stimulation

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

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