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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

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

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

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

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

CMT1A current gene therapy approaches and promising biomarkers

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Neural Regeneration Research 2023年第7期18卷 1434-1440页

作者： Marina Stavrou Kleopas AKleopa Neuroscience Department The Cyprus Institute of Neurology and GeneticsNicosiaCyprus Center for neuromuscular Disorders The Cyprus Institute of Neurology and GeneticsNicosiaCyprus

Charcot-Marie-Tooth neuropathies(CMT)constitute a group of common but highly heterogeneous,non-syndromic genetic disorders affecting predominantly the peripheral nervous *** type 1A(CMT1A)is the most frequent type and accounts for almost~50%of all diagnosed CMT ***1A results from the duplication of the peripheral myelin protein 22(PMP22)*** of PMP22 protein overloads the protein folding apparatus in Schwann cells and activates the unfolded protein *** leads to Schwann cell apoptosis,dys-and de-myelination and secondary axonal degeneration,ultimately causing neurological *** the last decades,several different gene therapies have been developed to treat *** all of them remain at the pre-clinical stage using CMT1A animal models overexpressing *** therapeutic goal is to achieve gene silencing,directly or indirectly,thereby reversing the CMT1A genetic mechanism allowing the recovery of myelination and prevention of axonal *** promising treatments are rapidly emerging,treatment-responsive and clinically relevant biomarkers are becoming *** biomarkers and sensitive clinical evaluation tools will facilitate the design and successful completion of future clinical trials for CMT1A.

关键词： axonal degeneration biomarkers Charcot-Marie-Tooth disease gene therapy inherited neuropathy mouse models

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

Effects of progesterone on T-type-Ca^(2+)-channel expression in Purkinje cells

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Neural Regeneration Research 2022年第11期17卷 2465-2471页

作者： Annika Eickhoff Jonas Tjaden Sarah Stahlke Matthias Vorgerd Verena Theis Veronika Matschke Carsten Theiss Department of Cytology Institute of AnatomyRuhr-University BochumBochumGermany Department of Neurology Neuromuscular Center RuhrgebietUniversity Hospital BergmannsheilRuhr-Universität BochumBochumGermany

Plasticity of cerebellar Purkinje cells(PC)is influenced by progeste rone via the classical progeste rone receptors PR-A and PR-B by stimulating dendritogenesis,spinogenesis,and synaptogenesis in these *** PC cultures were used to analyze progeste rone effects at a molecular level on the voltage-gated T-type-Ca^(2+)-channels Ca_(v)3.1,Ca_(v)3.2,and Ca_(v)3.3 as they helped determine neuronal plasticity by regulating Ca^(2+)-influx in neuronal *** results showed direct effects of progesterone on the mRNA expression of T-type-Ca^(2+)-channels,as well as on the protein kinases A and C being involved in downstream signaling pathways that play an important role in neuronal *** the mRNA expression studies of T-type-Ca^(2+)-channels and protein kinases of the signaling cascade,laser microdissection and purified PC cultures of diffe rent maturation stages were *** staining was also performed to characte rize the localization of T-type-Ca^(2+)-channels in *** rimental progesterone treatment was performed on the purified PC culture for 24 and 48 *** results show that progesterone increases the expression of Ca_(v)3.1 and Ca_(v)3.3 and associated protein kinases A and Cin PC at the mRNA level within 48 hours after treatment at *** effects extend the current knowledge of the function of progesterone in the central nervous system and provide an explanatory approach for its influence on neuronal plasticity.

关键词： Ca_(v)3.1 Ca_(v)3.2 Ca_(v)3.3 neuronal plasticity progesterone protein kinase A protein kinase C Purkinje cells rat cerebellum T-type-Ca^(2+)-channels