Related references
Note: Only part of the references are listed.Sarcolipin haploinsufficiency prevents dystrophic cardiomyopathy in mdx mice
Satvik Mareedu et al.
AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY (2021)
Roles of cytokines and T cells in the pathogenesis of myasthenia gravis
A. Uzawa et al.
CLINICAL AND EXPERIMENTAL IMMUNOLOGY (2021)
Mesenchymal Bmp3b expression maintains skeletal muscle integrity and decreases in age-related sarcopenia
Akiyoshi Uezumi et al.
JOURNAL OF CLINICAL INVESTIGATION (2021)
Modeling CNS Involvement in Pompe Disease Using Neural Stem Cells Generated from Patient-Derived Induced Pluripotent Stem Cells
Yu-Shan Cheng et al.
CELLS (2021)
iPSC-derived cardiomyocytes from patients with myotonic dystrophy type 1 have abnormal ion channel functions and slower conduction velocities
Hugo Poulin et al.
SCIENTIFIC REPORTS (2021)
Tissue-Engineered Skeletal Muscle Models to Study Muscle Function, Plasticity, and Disease
Alastair Khodabukus
FRONTIERS IN PHYSIOLOGY (2021)
Therapeutic efficacy of 3,4-Diaminopyridine phosphate on neuromuscular junction in Pompe disease
Bragato Cinzia et al.
BIOMEDICINE & PHARMACOTHERAPY (2021)
Myotonic dystrophy type 1 drug development: A pipeline toward the market
Marta Pascual-Gilabert et al.
DRUG DISCOVERY TODAY (2021)
Bioengineered in vitro 3D model of myotonic dystrophy type 1 human skeletal muscle
Xiomara Fernandez-Garibay et al.
BIOFABRICATION (2021)
Characterization of hiPSC-Derived Muscle Progenitors Reveals Distinctive Markers for Myogenic Cell Purification Toward Cell Therapy
Minas Nalbandian et al.
STEM CELL REPORTS (2021)
Temporal mechanisms of myogenic specification in human induced pluripotent stem cells
P. Nayak et al.
SCIENCE ADVANCES (2021)
Precise correction of Duchenne muscular dystrophy exon deletion mutations by base and prime editing
F. Chemello et al.
SCIENCE ADVANCES (2021)
Three-dimensional tissue-engineered human skeletal muscle model of Pompe disease
Jason Wang et al.
COMMUNICATIONS BIOLOGY (2021)
De novo revertant fiber formation and therapy testing in a 3D culture model of Duchenne muscular dystrophy skeletal muscle
Majid Ebrahimi et al.
ACTA BIOMATERIALIA (2021)
Bioengineered optogenetic model of human neuromuscular junction
Olaia F. Vila et al.
BIOMATERIALS (2021)
Full-length dystrophin restoration via targeted exon integration by AAV-CRISPR in a humanized mouse model of Duchenne muscular dystrophy
Adrian Pickar-Oliver et al.
MOLECULAR THERAPY (2021)
Prednisolone rescues Duchenne muscular dystrophy phenotypes in human pluripotent stem cell-derived skeletal muscle in vitro
Ziad Al Tanoury et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2021)
Bioengineered model of the human motor unit with physiologically functional neuromuscular junctions
Rowan P. Rimington et al.
SCIENTIFIC REPORTS (2021)
Optogenetic modeling of human neuromuscular circuits in Duchenne muscular dystrophy with CRISPR and pharmacological corrections
Amaia Paredes-Redondo et al.
SCIENCE ADVANCES (2021)
A 3D disease and regeneration model of peripheral nervous system-on-a-chip
Sujin Hyung et al.
SCIENCE ADVANCES (2021)
The use of genetically humanized animal models for personalized medicine approaches
Annemieke Aartsma-Rus et al.
DISEASE MODELS & MECHANISMS (2020)
Electrical Muscle Stimulation Accelerates Functional Recovery After Nerve Injury
Tengfei Fu et al.
NEUROSCIENCE (2020)
Self-Organizing 3D Human Trunk Neuromuscular Organoids
Jorge-Miguel Faustino Martins et al.
CELL STEM CELL (2020)
Mutant C9orf72 human iPSC-derived astrocytes cause non-cell autonomous motor neuron pathophysiology
Chen Zhao et al.
GLIA (2020)
Robotic fluidic coupling and interrogation of multiple vascularized organ chips
Richard Novak et al.
NATURE BIOMEDICAL ENGINEERING (2020)
Glycogen storage in a zebrafish Pompe disease model is reduced by 3-BrPA treatment
Cinzia Bragato et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE (2020)
A Human Skeletal Muscle Atlas Identifies the Trajectories of Stem and Progenitor Cells across Development and from Human Pluripotent Stem Cells
Haibin Xi et al.
CELL STEM CELL (2020)
A 96-well culture platform enables longitudinal analyses of engineered human skeletal muscle microtissue strength
Mohammad E. Afshar et al.
SCIENTIFIC REPORTS (2020)
Myogenic Progenitor Cell Lineage Specification by CRISPR/Cas9-Based Transcriptional Activators
Jennifer B. Kwon et al.
STEM CELL REPORTS (2020)
Multilineage Differentiation for Formation of Innervated Skeletal Muscle Fibers from Healthy and Diseased Human Pluripotent Stem Cells
Kilian Mazaleyrat et al.
CELLS (2020)
Duchenne muscular dystrophy hiPSC-derived myoblast drug screen identifies compounds that ameliorate disease in mdx mice
Congshan Sun et al.
JCI INSIGHT (2020)
Pre-innervated tissue-engineered muscle promotes a pro-regenerative microenvironment following volumetric muscle loss
Suradip Das et al.
COMMUNICATIONS BIOLOGY (2020)
Self-Organizing 3D Human Trunk Neuromuscular Organoids (vol 26, pg 172, 2020)
Jorge-Miguel Faustino Martins et al.
CELL STEM CELL (2020)
Global epidemiology of Duchenne muscular dystrophy: an updated systematic review and meta-analysis
Salvatore Crisafulli et al.
ORPHANET JOURNAL OF RARE DISEASES (2020)
Properties of Glial Cell at the Neuromuscular Junction Are Incompatible with Synaptic Repair in the SOD1G37R ALS Mouse Model
Eric Martineau et al.
JOURNAL OF NEUROSCIENCE (2020)
Functional skeletal muscle model derived from SOD1-mutant ALS patient iPSCs recapitulates hallmarks of disease progression
Agnes Badu-Mensah et al.
SCIENTIFIC REPORTS (2020)
Single-nucleus RNA-seq identifies transcriptional heterogeneity in multinucleated skeletal myofibers
Michael J. Petrany et al.
NATURE COMMUNICATIONS (2020)
Human myotube formation is determined by MyoD-Myomixer/Myomaker axis
Haifeng Zhang et al.
SCIENCE ADVANCES (2020)
The Failed Clinical Story of Myostatin Inhibitors against Duchenne Muscular Dystrophy: Exploring the Biology behind the Battle
Emma Rybalka et al.
CELLS (2020)
Gene therapy with secreted acid alpha-glucosidase rescues Pompe disease in a novel mouse model with early-onset spinal cord and respiratory defects
Pasqualina Colella et al.
EBIOMEDICINE (2020)
Characterization of Functional Human Skeletal Myotubes and Neuromuscular Junction Derived-From the Same Induced Pluripotent Stem Cell Source
Xiufang Guo et al.
BIOENGINEERING-BASEL (2020)
A Human-Based Functional NMJ System for Personalized ALS Modeling and Drug Testing
Xiufang Guo et al.
ADVANCED THERAPEUTICS (2020)
Electrical stimulation increases hypertrophy and metabolic flux in tissue-engineered human skeletal muscle
Alastair Khodabukus et al.
BIOMATERIALS (2019)
Riluzole and edaravone: A tale of two amyotrophic lateral sclerosis drugs
Manoj Kumar Jaiswal
MEDICINAL RESEARCH REVIEWS (2019)
EGFR-Aurka Signaling Rescues Polarity and Regeneration Defects in Dystrophin-Deficient Muscle Stem Cells by Increasing Asymmetric Divisions
Yu Xin Wang et al.
CELL STEM CELL (2019)
Gene therapies in canine models for Duchenne muscular dystrophy
Peter P. Nghiem et al.
HUMAN GENETICS (2019)
CRISPR-Cas9 corrects Duchenne muscular dystrophy exon 44 deletion mutations in mice and human cells
Yi-Li Min et al.
SCIENCE ADVANCES (2019)
Micropatterned substrates with physiological stiffness promote cell maturation and Pompe disease phenotype in human induced pluripotent stem cell-derived skeletal myocytes
Nunnapas Jiwlawat et al.
BIOTECHNOLOGY AND BIOENGINEERING (2019)
Natural disease history of the D2-mdx mouse model for Duchenne muscular dystrophy
Maaike van Putten et al.
FASEB JOURNAL (2019)
Comparison of three congruent patient-specific cell types for the modelling of a human genetic Schwann-cell disorder
Bipasha Mukherjee-Clavin et al.
NATURE BIOMEDICAL ENGINEERING (2019)
Optogenetic neuronal stimulation promotes axon outgrowth and myelination of motor neurons in a three-dimensional motor neuron-Schwann cell coculture model on a microfluidic biochip
Sujin Hyung et al.
BIOTECHNOLOGY AND BIOENGINEERING (2019)
Fibroadipogenic progenitors are responsible for muscle loss in limb girdle muscular dystrophy 2B
Marshall W. Hogarth et al.
NATURE COMMUNICATIONS (2019)
C9ORF72-related cellular pathology in skeletal myocytes derived from ALS-patient induced pluripotent stem cells
Eileen Lynch et al.
DISEASE MODELS & MECHANISMS (2019)
Using human Pompe disease-induced pluripotent stem cell-derived neural cells to identify compounds with therapeutic potential
Hsiang-Po Huang et al.
HUMAN MOLECULAR GENETICS (2019)
Dystrophin deficiency leads to dysfunctional glutamate clearance in iPSC derived astrocytes
Abdulsamie M. Patel et al.
TRANSLATIONAL PSYCHIATRY (2019)
AAV Gene Therapy Utilizing Glycosylation-Independent Lysosomal Targeting Tagged GAA in the Hypoglossal Motor System of Pompe Mice
Brendan M. Doyle et al.
MOLECULAR THERAPY-METHODS & CLINICAL DEVELOPMENT (2019)
Engineered skeletal muscles for disease modeling and drug discovery
Jason Wang et al.
BIOMATERIALS (2019)
Biomaterials and Advanced Biofabrication Techniques in hiPSCs Based Neuromyopathic Disease Modeling
Jing Sun et al.
FRONTIERS IN BIOENGINEERING AND BIOTECHNOLOGY (2019)
Examining the relationship between astrocyte dysfunction and neurodegeneration in ALS using hiPSCs
Madeline Halpern et al.
NEUROBIOLOGY OF DISEASE (2019)
DP71 and SERCA2 alteration in human neurons of a Duchenne muscular dystrophy patient
Simona Ruggieri et al.
STEM CELL RESEARCH & THERAPY (2019)
Human iPSC-derived astrocytes from ALS patients with mutated C9ORF72 show increased oxidative stress and neurotoxicity
Anastasya Birger et al.
EBioMedicine (2019)
Quantification of human neuromuscular function through optogenetics
Olaia F. Vila et al.
THERANOSTICS (2019)
iPSC-derived functional human neuromuscular junctions model the pathophysiology of neuromuscular diseases
Chuang-Yu Lin et al.
JCI INSIGHT (2019)
Time-Dependent Addition of Neuronal and Schwann Cells Increase Myotube Viability and Length in an In Vitro Tri-culture Model of the Neuromuscular Junction
Tanya Singh et al.
REGENERATIVE ENGINEERING AND TRANSLATIONAL MEDICINE (2019)
Eteplirsen Treatment Attenuates Respiratory Decline in Ambulatory and Non-Ambulatory Patients with Duchenne Muscular Dystrophy
Navid Khan et al.
JOURNAL OF NEUROMUSCULAR DISEASES (2019)
A Neuron-Specific Gene Therapy Relieves Motor Deficits in Pompe Disease Mice
Ni-Chung Lee et al.
MOLECULAR NEUROBIOLOGY (2018)
ERBB3 and NGFR mark a distinct skeletal muscle progenitor cell in human development and hPSCs
Michael R. Hicks et al.
NATURE CELL BIOLOGY (2018)
Collagen VI is required for the structural and functional integrity of the neuromuscular junction
Matilde Cescon et al.
ACTA NEUROPATHOLOGICA (2018)
Neuromuscular Junction Formation, Aging, and Disorders
Lei Li et al.
ANNUAL REVIEW OF PHYSIOLOGY, VOL 80 (2018)
Stem cell derived phenotypic human neuromuscular junction model for dose response evaluation of therapeutics
Navaneetha Santhanam et al.
BIOMATERIALS (2018)
The Dystrophin Glycoprotein Complex Regulates the Epigenetic Activation of Muscle Stem Cell Commitment
Natasha C. Chang et al.
CELL STEM CELL (2018)
Tracking progress: an update on animal models for Duchenne muscular dystrophy
Dominic J. Wells
DISEASE MODELS & MECHANISMS (2018)
A novel rabbit model of Duchenne muscular dystrophy generated by CRISPR/Cas9
Tingting Sui et al.
DISEASE MODELS & MECHANISMS (2018)
Animal Models of the Neuromuscular Junction, Vitally Informative for Understanding Function and the Molecular Mechanisms of Congenital Myasthenic Syndromes
Richard G. Webster
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2018)
Collagen XIII Is Required for Neuromuscular Synapse Regeneration and Functional Recovery after Peripheral Nerve Injury
Zarin Zainul et al.
JOURNAL OF NEUROSCIENCE (2018)
Amyotrophic Lateral Sclerosis: An Update for 2018
Bjorn Oskarsson et al.
MAYO CLINIC PROCEEDINGS (2018)
Denervation-activated STAT3-IL-6 signalling in fibro-adipogenic progenitors promotes myofibres atrophy and fibrosis
Luca Madaro et al.
NATURE CELL BIOLOGY (2018)
Haploinsufficiency leads to neurodegeneration in C9ORF72 ALS/FTD human induced motor neurons
Yingxiao Shi et al.
NATURE MEDICINE (2018)
Treatment of Myasthenia Gravis
Constantine Farmakidis et al.
NEUROLOGIC CLINICS (2018)
Introducing Autoimmunity at the Synapse by a Novel Animal Model of Experimental Autoimmune Myasthenia Gravis
Jianwen Wang et al.
NEUROSCIENCE (2018)
A dystrophic Duchenne mouse model for testing human antisense oligonucleotides
Marcel Veltrop et al.
PLOS ONE (2018)
Lymphoblastoids cell lines - Derived iPSC line from a 26-year-old myotonic dystrophy type 1 patient carrying (CTG)(200) expansion in the DMPK gene: CHUQi001-A
Laurie Martineau et al.
STEM CELL RESEARCH (2018)
Engineering human pluripotent stem cells into a functional skeletal muscle tissue
Lingjun Rao et al.
NATURE COMMUNICATIONS (2018)
3D bio-printed scaffold-free nerve constructs with human gingiva-derived mesenchymal stem cells promote rat facial nerve regeneration
Qunzhou Zhang et al.
SCIENTIFIC REPORTS (2018)
Three-Dimensional Human iPSC-Derived Artificial Skeletal Muscles Model Muscular Dystrophies and Enable Multilineage Tissue Engineering
Sara Martina Maffioletti et al.
CELL REPORTS (2018)
Current Progress and Challenges for Skeletal Muscle Differentiation from Human Pluripotent Stem Cells Using Transgene-Free Approaches
Nunnapas Jiwlawat et al.
STEM CELLS INTERNATIONAL (2018)
Large-Scale Expansion of Human iPSC-Derived Skeletal Muscle Cells for Disease Modeling and Cell-Based Therapeutic Strategies
Erik van der Wal et al.
STEM CELL REPORTS (2018)
Cells of Matter-In Vitro Models for Myotonic Dystrophy
Magdalena Matloka et al.
FRONTIERS IN NEUROLOGY (2018)
Correction of diverse muscular dystrophy mutations in human engineered heart muscle by single-site genome editing
Chengzu Long et al.
SCIENCE ADVANCES (2018)
Recovery of respiratory function in mdx mice co-treated with neutralizing interleukin-6 receptor antibodies and urocortin-2
David P. Burns et al.
JOURNAL OF PHYSIOLOGY-LONDON (2018)
Enhanced skeletal muscle formation on microfluidic spun gelatin methacryloyl (GelMA) fibres using surface patterning and agrin treatment
Majid Ebrahimi et al.
JOURNAL OF TISSUE ENGINEERING AND REGENERATIVE MEDICINE (2018)
Modeling sporadic ALS in iPSC-derived motor neurons identifies a potential therapeutic agent
Koki Fujimori et al.
NATURE MEDICINE (2018)
Animal models of neurodegenerative diseases
Ted M. Dawson et al.
NATURE NEUROSCIENCE (2018)
Smart Biomaterials: Recent Advances and Future Directions
Piotr S. Kowalski et al.
ACS BIOMATERIALS SCIENCE & ENGINEERING (2018)
Incorporation of macrophages into engineered skeletal muscle enables enhanced muscle regeneration
Mark Juhas et al.
NATURE BIOMEDICAL ENGINEERING (2018)
Dystrophin As a Molecular Shock Absorber
Shimin Le et al.
ACS NANO (2018)
Development of an innervated tissue-engineered skin with human sensory neurons and Schwann cells differentiated from iPS cells
Quentin Muller et al.
ACTA BIOMATERIALIA (2018)
AMPK Activation Regulates LTBP4-Dependent TGF-beta 1 Secretion by Pro-inflammatory Macrophages and Controls Fibrosis in Duchenne Muscular Dystrophy
Gaetan Juban et al.
CELL REPORTS (2018)
Engineering an Environment for the Study of Fibrosis: A 3D Human Muscle Model with Endothelium Specificity and Endomysium
Simone Bersini et al.
CELL REPORTS (2018)
Microphysiological 3D model of amyotrophic lateral sclerosis (ALS) from human iPS-derived muscle cells and optogenetic motor neurons
Tatsuya Osaki et al.
SCIENCE ADVANCES (2018)
Pompe disease in adulthood: effects of antibody formation on enzyme replacement therapy
Juna M. de Vries et al.
GENETICS IN MEDICINE (2017)
Opposite Synaptic Alterations at the Neuromuscular Junction in an ALS Mouse Model: When Motor Units Matter
Elsa Tremblay et al.
JOURNAL OF NEUROSCIENCE (2017)
The role of laminins in the organization and function of neuromuscular junctions
Robert S. Rogers et al.
MATRIX BIOLOGY (2017)
Mechanically patterned neuromuscular junctions-in-a-dish have improved functional maturation
Cassandra L. Happe et al.
MOLECULAR BIOLOGY OF THE CELL (2017)
Cognitive and academic outcomes in long-term survivors of infantile-onset Pompe disease: A longitudinal follow-up
Gail A. Spiridigliozzi et al.
MOLECULAR GENETICS AND METABOLISM (2017)
The emerging phenotype of late-onset Pompe disease: A systematic literature review
Justin Chan et al.
MOLECULAR GENETICS AND METABOLISM (2017)
Leveraging advances in biology to design biomaterials
Max Darnell et al.
NATURE MATERIALS (2017)
The role of peripheral immune cells in the CNS in steady state and disease
Marco Prinz et al.
NATURE NEUROSCIENCE (2017)
Receptor and post-receptor abnormalities contribute to insulin resistance in myotonic dystrophy type 1 and type 2 skeletal muscle
Laura Valentina Renna et al.
PLOS ONE (2017)
Human endothelial cells secrete neurotropic factors to direct axonal growth of peripheral nerves
Jonathan M. Grasman et al.
SCIENTIFIC REPORTS (2017)
Downregulation of the Glial GLT1 Glutamate Transporter and Purkinje Cell Dysfunction in a Mouse Model of Myotonic Dystrophy
Geraldine Sicot et al.
CELL REPORTS (2017)
Aberrant Myokine Signaling in Congenital Myotonic Dystrophy
Masayuki Nakamori et al.
CELL REPORTS (2017)
Cellular and Molecular Anatomy of the Human Neuromuscular Junction
Ross A. Jones et al.
CELL REPORTS (2017)
Amyotrophic lateral sclerosis patient iPSC-derived astrocytes impair autophagy via non-cell autonomous mechanisms
Martin Madill et al.
MOLECULAR BRAIN (2017)
Skeletal Muscle Cell Induction from Pluripotent Stem Cells
Yusaku Kodaka et al.
STEM CELLS INTERNATIONAL (2017)
Measuring Neuromuscular Junction Functionality
Emanuele Rizzuto et al.
JOVE-JOURNAL OF VISUALIZED EXPERIMENTS (2017)
GAA Deficiency in Pompe Disease Is Alleviated by Exon Inclusion in iPSC-Derived Skeletal Muscle Cells
Erik van der Wal et al.
MOLECULAR THERAPY-NUCLEIC ACIDS (2017)
Schwann Cell Precursors from Human Pluripotent Stem Cells as a Potential Therapeutic Target for Myelin Repair
Han-Seop Kim et al.
STEM CELL REPORTS (2017)
ALS skeletal muscle shows enhanced TGF-β signaling, fibrosis and induction of fibro/adipogenic progenitor markers
David Gonzalez et al.
PLOS ONE (2017)
Drosophila studies support a role for a presynaptic synaptotagmin mutation in a human congenital myasthenic syndrome
Mallory C. Shields et al.
PLOS ONE (2017)
Myotonic dystrophy type 1 patient-derived iPSCs for the investigation of CTG repeat instability
Junko Ueki et al.
SCIENTIFIC REPORTS (2017)
Multi-tissue interactions in an integrated three-tissue organ-on-a-chip platform
Aleksander Skardal et al.
SCIENTIFIC REPORTS (2017)
AAV-mediated transcription factor EB (TFEB) gene delivery ameliorates muscle pathology and function in the murine model of Pompe Disease
Francesca Gatto et al.
SCIENTIFIC REPORTS (2017)
The Differentiation Stage of Transplanted Stem Cells Modulates Nerve Regeneration
Ching-Wen Huang et al.
SCIENTIFIC REPORTS (2017)
A Skeletal Muscle Model of Infantile-onset Pompe Disease with Patient-specific iPS Cells
Takeshi Yoshida et al.
SCIENTIFIC REPORTS (2017)
Myotonic dystrophy: candidate small molecule therapeutics
Piotr Konieczny et al.
DRUG DISCOVERY TODAY (2017)
Muscle satellite cells are functionally impaired in myasthenia gravis: consequences on muscle regeneration
Mohamed Attia et al.
ACTA NEUROPATHOLOGICA (2017)
Evidence for mast cells contributing to neuromuscular pathology in an inherited model of ALS
Emiliano Trias et al.
JCI INSIGHT (2017)
Functional correction of dystrophin actin binding domain mutations by genome editing
Viktoriia Kyrychenko et al.
JCI INSIGHT (2017)
Wnt proteins contribute to neuromuscular junction formation through distinct signaling pathways
Julien Messeant et al.
DEVELOPMENT (2017)
A Gene Regulatory Network Balances Neural and Mesoderm Specification during Vertebrate Trunk Development
Mina Gouti et al.
DEVELOPMENTAL CELL (2017)
Neurodevelopmental, emotional, and behavioural problems in Duchenne muscular dystrophy in relation to underlying dystrophin gene mutations
Valeria Ricotti et al.
DEVELOPMENTAL MEDICINE AND CHILD NEUROLOGY (2016)
Combination of agrin and laminin increase acetylcholine receptor clustering and enhance functional neuromuscular junction formation In vitro
Bill G. X. Zhang et al.
DEVELOPMENTAL NEUROBIOLOGY (2016)
The Role of Skeletal Muscle in Amyotrophic Lateral Sclerosis
Jean-Philippe Loeffler et al.
BRAIN PATHOLOGY (2016)
Functional Connectivity under Optogenetic Control Allows Modeling of Human Neuromuscular Disease
Julius A. Steinbeck et al.
CELL STEM CELL (2016)
Neuromuscular transmission abnormalities in myotonic dystrophy type 1: A neurophysiological study
Francesco Bombelli et al.
CLINICAL NEUROLOGY AND NEUROSURGERY (2016)
A system for studying mechanisms of neuromuscular junction development and maintenance
Valerie Vilmont et al.
DEVELOPMENT (2016)
An NF-κB-EphrinA5-Dependent Communication between NG2+ Interstitial Cells and Myoblasts Promotes Muscle Growth in Neonates
Jin-Mo Gu et al.
DEVELOPMENTAL CELL (2016)
Compartmental microfluidic system for studying muscle-neuron communication and neuromuscular junction maintenance
Ariel Ionescu et al.
EUROPEAN JOURNAL OF CELL BIOLOGY (2016)
Characterization of neuromuscular synapse function abnormalities in multiple Duchenne muscular dystrophy mouse models
Elizabeth M. van der Pijl et al.
EUROPEAN JOURNAL OF NEUROSCIENCE (2016)
Neuromuscular junction immaturity and muscle atrophy are hallmarks of the ColQ-deficient mouse, a model of congenital myasthenic syndrome with acetylcholinesterase deficiency
Severine M. Sigoillot et al.
FASEB JOURNAL (2016)
Long-term, high-level hepatic secretion of acid α-glucosidase for Pompe disease achieved in non- human primates using helper-dependent adenovirus
D. P. W. Rastall et al.
GENE THERAPY (2016)
A human in vitro model of Duchenne muscular dystrophy muscle formation and contractility
Alexander P. Nesmith et al.
JOURNAL OF CELL BIOLOGY (2016)
Schwann Cells in Neuromuscular Junction Formation and Maintenance
Arnab Barik et al.
JOURNAL OF NEUROSCIENCE (2016)
Salmeterol enhances the cardiac response to gene therapy in Pompe disease
Sang-oh Han et al.
MOLECULAR GENETICS AND METABOLISM (2016)
Genome Therapy of Myotonic Dystrophy Type 1 iPS Cells for Development of Autologous Stem Cell Therapy
Yuanzheng Gao et al.
MOLECULAR THERAPY (2016)
A 3D bioprinting system to produce human-scale tissue constructs with structural integrity
Hyun-Wook Kang et al.
NATURE BIOTECHNOLOGY (2016)
Generation of human muscle fibers and satellite-like cells from human pluripotent stem cells in vitro
Jerome Chal et al.
NATURE PROTOCOLS (2016)
Neuropathology in respiratory-related motoneurons in young Pompe (Gaa-/-) mice
Sara M. F. Turner et al.
RESPIRATORY PHYSIOLOGY & NEUROBIOLOGY (2016)
Differentiation of control and ALS mutant human iPSCs into functional skeletal muscle cells, a tool for the study of neuromuscolar diseases
Jessica Lenzi et al.
STEM CELL RESEARCH (2016)
Micropatterning Facilitates the Long-Term Growth and Analysis of iPSC-Derived Individual Human Neurons and Neuronal Networks
Lena F. Burbulla et al.
ADVANCED HEALTHCARE MATERIALS (2016)
A Novel Protocol for Directed Differentiation of C9orf72-Associated Human Induced Pluripotent Stem Cells Into Contractile Skeletal Myotubes
Elliot W. Swartz et al.
STEM CELLS TRANSLATIONAL MEDICINE (2016)
TFEB overexpression promotes glycogen clearance of Pompe disease iPSC-derived skeletal muscle
Yohei Sato et al.
MOLECULAR THERAPY-METHODS & CLINICAL DEVELOPMENT (2016)
Microfluidic device for the formation of optically excitable, three-dimensional, compartmentalized motor units
Sebastien G. M. Uzel et al.
SCIENCE ADVANCES (2016)
Prolonged Culture of Aligned Skeletal Myotubes on Micromolded Gelatin Hydrogels
Archana Bettadapur et al.
SCIENTIFIC REPORTS (2016)
Animal models of myasthenia gravis: utility and limitations
Renato Mantegazza et al.
INTERNATIONAL JOURNAL OF GENERAL MEDICINE (2016)
Correcting Neuromuscular Deficits With Gene Therapy in Pompe Disease
Adrian G. Todd et al.
ANNALS OF NEUROLOGY (2015)
Complicating autoimmune diseases in myasthenia gravis: a review
Aliona Nacu et al.
AUTOIMMUNITY (2015)
Myasthenia gravis and related disorders: Pathology and molecular pathogenesis
James C. Ha et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE (2015)
Lineage tracing of neuromesodermal progenitors reveals novel Wnt-dependent roles in trunk progenitor cell maintenance and differentiation
Robert J. Garriock et al.
DEVELOPMENT (2015)
Intrinsic and extrinsic mechanisms regulating satellite cell function
Nicolas A. Dumon et al.
DEVELOPMENT (2015)
Modeling and study of the mechanism of dilated cardiomyopathy using induced pluripotent stem cells derived from individuals with Duchenne muscular dystrophy
Bo Lin et al.
DISEASE MODELS & MECHANISMS (2015)
Motor neurons and oligodendrocytes arise from distinct cell lineages by progenitor recruitment
Andrew M. Ravanelli et al.
GENES & DEVELOPMENT (2015)
Porcine Models of Muscular Dystrophy
Joshua T. Selsby et al.
ILAR JOURNAL (2015)
What has the mdx mouse model of duchenne muscular dystrophy contributed to our understanding of this disease?
Jennifer Manning et al.
JOURNAL OF MUSCLE RESEARCH AND CELL MOTILITY (2015)
Duchenne muscular dystrophy
Eppie M. Yiu et al.
JOURNAL OF PAEDIATRICS AND CHILD HEALTH (2015)
The C9orf72 repeat expansion disrupts nucleocytoplasmic transport
Ke Zhang et al.
NATURE (2015)
Dystrophin expression in muscle stem cells regulates their polarity and asymmetric division
Nicolas A. Dumont et al.
NATURE MEDICINE (2015)
Efficient derivation and inducible differentiation of expandable skeletal myogenic cells from human ES and patient-specific iPS cells
Sara M. Maffioletti et al.
NATURE PROTOCOLS (2015)
Progressive Muscular Atrophy
Teerin Liewluck et al.
NEUROLOGIC CLINICS (2015)
Impaired autophagy affects acid alpha-glucosidase processing and enzyme replacement therapy efficacy in late-onset glycogen storage disease type II
A. C. Nascimbeni et al.
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY (2015)
MECHANISMS REGULATING NEUROMUSCULAR JUNCTION DEVELOPMENT AND FUNCTION AND CAUSES OF MUSCLE WASTING
Lionel A. Tintignac et al.
PHYSIOLOGICAL REVIEWS (2015)
Formation and characterisation of neuromuscular junctions between hiPSC derived motoneurons and myotubes
M. Demestre et al.
STEM CELL RESEARCH (2015)
Genome Modification Leads to Phenotype Reversal in Human Myotonic Dystrophy Type 1 Induced Pluripotent Stem Cell-Derived Neural Stem Cells
Guangbin Xia et al.
STEM CELLS (2015)
Neuromuscular Junction Formation in Tissue-Engineered Skeletal Muscle Augments Contractile Function and Improves Cytoskeletal Organization
Neil R. W. Martin et al.
TISSUE ENGINEERING PART A (2015)
Activation of the endoplasmic reticulum stress response in skeletal muscle of G93A*SOD1 amyotrophic lateral sclerosis mice
Dapeng Chen et al.
FRONTIERS IN CELLULAR NEUROSCIENCE (2015)
Perisynaptic Schwann Cells at the Neuromuscular Synapse: Adaptable, Multitasking Glial Cells
Chien-Ping Ko et al.
COLD SPRING HARBOR PERSPECTIVES IN BIOLOGY (2015)
Interactions between muscle stem cells, mesenchymal-derived cells and immune cells in muscle homeostasis, regeneration and disease
J. Farup et al.
CELL DEATH & DISEASE (2015)
Generation and expansion of highly pure motor neuron progenitors from human pluripotent stem cells
Zhong-Wei Du et al.
NATURE COMMUNICATIONS (2015)
Pre- and postsynaptic changes in the neuromuscular junction in dystrophic mice
Stephen J. P. Pratt et al.
FRONTIERS IN PHYSIOLOGY (2015)
In Vitro and In Vivo Modulation of Alternative Splicing by the Biguanide Metformin
Delphine Laustriat et al.
MOLECULAR THERAPY-NUCLEIC ACIDS (2015)
Modeling the Early Phenotype at the Neuromuscular Junction of Spinal Muscular Atrophy Using Patient-Derived iPSCs
Michiko Yoshida et al.
STEM CELL REPORTS (2015)
IL-6 and Akt are involved in muscular pathogenesis in myasthenia gravis
Marie Maurer et al.
ACTA NEUROPATHOLOGICA COMMUNICATIONS (2015)
Recovery of altered neuromuscular junction morphology and muscle function in mdx mice after injury
Stephen J. P. Pratt et al.
CELLULAR AND MOLECULAR LIFE SCIENCES (2015)
TWEAK/Fn14, a pathway and novel therapeutic target in myotonic dystrophy
Ramesh S. Yadava et al.
HUMAN MOLECULAR GENETICS (2015)
Peripheral nerve and neuromuscular junction pathology in Pompe disease
Darin J. Falk et al.
HUMAN MOLECULAR GENETICS (2015)
Combinatorial analysis of developmental cues efficiently converts human pluripotent stem cells into multiple neuronal subtypes
Yves Maury et al.
NATURE BIOTECHNOLOGY (2015)
Coculture of Primary Motor Neurons and Schwann Cells as a Model for In Vitro Myelination
Sujin Hyung et al.
SCIENTIFIC REPORTS (2015)
Endplate denervation correlates with Nogo-A muscle expression in amyotrophic lateral sclerosis patients
Gaelle Bruneteau et al.
ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY (2015)
Cortactin autoantibodies in myasthenia gravis
Eduard Gallardo et al.
AUTOIMMUNITY REVIEWS (2014)
Roles of adherent myogenic cells and dynamic culture in engineered muscle function and maintenance of satellite cells
Mark Juhas et al.
BIOMATERIALS (2014)
Pathways Disrupted in Human ALS Motor Neurons Identified through Genetic Correction of Mutant SOD1
Evangelos Kiskinis et al.
CELL STEM CELL (2014)
Generating spinal motor neuron diversity: a long quest for neuronal identity
Cedric Francius et al.
CELLULAR AND MOLECULAR LIFE SCIENCES (2014)
Early Gene Expression Changes in Skeletal Muscle from SOD1G93A Amyotrophic Lateral Sclerosis Animal Model
Gabriela P. de Oliveira et al.
CELLULAR AND MOLECULAR NEUROBIOLOGY (2014)
Mesogenin 1 is a master regulator of paraxial presomitic mesoderm differentiation
Ravindra B. Chalamalasetty et al.
DEVELOPMENT (2014)
A three-dimensional human neural cell culture model of Alzheimer's disease
Se Hoon Choi et al.
NATURE (2014)
Anti-agrin autoantibodies in myasthenia gravis
Christiane Gasperi et al.
NEUROLOGY (2014)
A Quantitative Framework to Evaluate Modeling of Cortical Development by Neural Stem Cells
Jason L. Stein et al.
NEURON (2014)
Genotype-phenotype correlation in Pompe disease, a step forward
Paola De Filippi et al.
ORPHANET JOURNAL OF RARE DISEASES (2014)
Characterization of Dystrophin Deficient Rats: A New Model for Duchenne Muscular Dystrophy
Thibaut Larcher et al.
PLOS ONE (2014)
Physiological and ultrastructural features of human induced pluripotent and embryonic stem cell-derived skeletal myocytes in vitro
Gunnar Skoglund et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2014)
Zebrafish models of human motor neuron diseases: Advantages and limitations
Patrick J. Babin et al.
PROGRESS IN NEUROBIOLOGY (2014)
Dlk1 Promotes a Fast Motor Neuron Biophysical Signature Required for Peak Force Execution
Daniel Mueller et al.
SCIENCE (2014)
Skeletal Muscle Satellite Cells in Amyotrophic Lateral Sclerosis
Annarita Scaramozza et al.
ULTRASTRUCTURAL PATHOLOGY (2014)
The role of oxidative stress in degeneration of the neuromuscular junction in amyotrophic lateral sclerosis
Eveliina Pollari et al.
FRONTIERS IN CELLULAR NEUROSCIENCE (2014)
Adipocytes arise from multiple lineages that are heterogeneously and dynamically distributed
Joan Sanchez-Gurmaches et al.
NATURE COMMUNICATIONS (2014)
Engineering a functional neuro-muscular junction model in a chip
Ziqiu Tong et al.
RSC ADVANCES (2014)
Intrinsic Membrane Hyperexcitability of Amyotrophic Lateral Sclerosis Patient-Derived Motor Neurons
Brian J. Wainger et al.
CELL REPORTS (2014)
Pompe disease: from pathophysiology to therapy and back again
Jeong-A Lim et al.
FRONTIERS IN AGING NEUROSCIENCE (2014)
Myogenic Differentiation of Muscular Dystrophy-Specific Induced Pluripotent Stem Cells for Use in Drug Discovery
Ramzey Abujarour et al.
STEM CELLS TRANSLATIONAL MEDICINE (2014)
ALS as a distal axonopathy: molecular mechanisms affecting neuromuscular junction stability in the presymptomatic stages of the disease
Elizabeth B. Moloney et al.
FRONTIERS IN NEUROSCIENCE (2014)
Vascular regulation of adult neurogenesis under physiological and pathological conditions
Masato Sawada et al.
FRONTIERS IN NEUROSCIENCE (2014)
Generation of Myospheres From hESCs by Epigenetic Reprogramming
Sonia Albini et al.
JOVE-JOURNAL OF VISUALIZED EXPERIMENTS (2014)
The value of muscle biopsies in Pompe disease: identifying lipofuscin inclusions in juvenile-and adult-onset patients
Erin J. Feeney et al.
ACTA NEUROPATHOLOGICA COMMUNICATIONS (2014)
Reduction in hSOD1 copy number significantly impacts ALS phenotype presentation in G37R (line 29) mice: implications for the assessment of putative therapeutic agents
Pierre Zwiegers et al.
JOURNAL OF NEGATIVE RESULTS IN BIOMEDICINE (2014)
Optimizing the structure and contractility of engineered skeletal muscle thin films
Y. Sun et al.
ACTA BIOMATERIALIA (2013)
LTBP4 genotype predicts age of ambulatory loss in duchenne muscular dystrophy
Kevin M. Flanigan et al.
ANNALS OF NEUROLOGY (2013)
Three-dimensional neuron-muscle constructs with neuromuscular junctions
Yuya Morimoto et al.
BIOMATERIALS (2013)
Human iPSC-Based Modeling of Late-Onset Disease via Progerin-Induced Aging
Justine D. Miller et al.
CELL STEM CELL (2013)
Transcription factor EB (TFEB) is a new therapeutic target for Pompe disease
Carmine Spampanato et al.
EMBO MOLECULAR MEDICINE (2013)
Axonal degeneration in the peripheral nervous system: Implications for the pathogenesis of amyotrophic lateral sclerosis
Lindsey R. Fischer-Hayes et al.
EXPERIMENTAL NEUROLOGY (2013)
Length-dependent CTG.CAG triplet-repeat expansion in myotonic dystrophy patient-derived induced pluripotent stem cells
Jintang Du et al.
HUMAN MOLECULAR GENETICS (2013)
Microfluidic-primary culture model of the lower motor neuron-neuromuscular junction circuit
Katherine A. Southam et al.
JOURNAL OF NEUROSCIENCE METHODS (2013)
Effects of in vivo injury on the neuromuscular junction in healthy and dystrophic muscles
Stephen J. P. Pratt et al.
JOURNAL OF PHYSIOLOGY-LONDON (2013)
Role of telomere dysfunction in cardiac failure in Duchenne muscular dystrophy
Foteini Mourkioti et al.
NATURE CELL BIOLOGY (2013)
The neuroinflammatory role of Schwann cells in disease
Elke Ydens et al.
NEUROBIOLOGY OF DISEASE (2013)
Altered in vitro Proliferation of Mouse SOD1-G93A Skeletal Muscle Satellite Cells
Raquel Manzano et al.
NEURODEGENERATIVE DISEASES (2013)
Peripheral neuropathy in patients with myotonic dystrophy type 1
Stojan Peric et al.
NEUROLOGICAL RESEARCH (2013)
Impact of enzyme replacement therapy on survival in adults with Pompe disease: results from a prospective international observational study
Deniz Gungor et al.
ORPHANET JOURNAL OF RARE DISEASES (2013)
Skeletal muscle pathology of infantile Pompe disease during long-term enzyme replacement therapy
Sean N. Prater et al.
ORPHANET JOURNAL OF RARE DISEASES (2013)
Efficient and Reproducible Myogenic Differentiation from Human iPS Cells: Prospects for Modeling Miyoshi Myopathy In Vitro
Akihito Tanaka et al.
PLOS ONE (2013)
Early Changes of Neuromuscular Transmission in the SOD1(G93A) Mice Model of ALS Start Long before Motor Symptoms Onset
Mariana C. Rocha et al.
PLOS ONE (2013)
Neural induction and early patterning in vertebrates
Mohammad Zeeshan Ozair et al.
WILEY INTERDISCIPLINARY REVIEWS-DEVELOPMENTAL BIOLOGY (2013)
A functional system for high-content screening of neuromuscular junctions in vitro
S. T. Smith et al.
TECHNOLOGY (2013)
The roles of RGD and grooved topography in the adhesion, morphology, and differentiation of C2C12 skeletal myoblasts
Peng-Yuan Wang et al.
BIOTECHNOLOGY AND BIOENGINEERING (2012)
The role of autophagy in the pathogenesis of glycogen storage disease type II (GSDII)
A. C. Nascimbeni et al.
CELL DEATH AND DIFFERENTIATION (2012)
Human ES- and iPS-Derived Myogenic Progenitors Restore DYSTROPHIN and Improve Contractility upon Transplantation in Dystrophic Mice
Radbod Darabi et al.
CELL STEM CELL (2012)
Interaction of Wnt3a, Msgn1 and Tbx6 in neural versus paraxial mesoderm lineage commitment and paraxial mesoderm differentiation in the mouse embryo
Sonja Nowotschin et al.
DEVELOPMENTAL BIOLOGY (2012)
Exon skipping for nonsense mutations in Duchenne muscular dystrophy: too many mutations, too few patients?
Toshifumi Yokota et al.
EXPERT OPINION ON BIOLOGICAL THERAPY (2012)
The emerging phenotype of long-term survivors with infantile Pompe disease
Sean N. Prater et al.
GENETICS IN MEDICINE (2012)
Regulation of Schwann cell differentiation and proliferation by the Pax-3 transcription factor
Robin D. S. Doddrell et al.
GLIA (2012)
The RNA-binding protein Staufen1 is increased in DM1 skeletal muscle and promotes alternative pre-mRNA splicing
Aymeric Ravel-Chapuis et al.
JOURNAL OF CELL BIOLOGY (2012)
AchR-positive myasthenia gravis with MRI evidence of early muscle atrophy
Vasiliki Zouvelou et al.
JOURNAL OF CLINICAL NEUROSCIENCE (2012)
Motor Neuron Rescue in Spinal Muscular Atrophy Mice Demonstrates That Sensory-Motor Defects Are a Consequence, Not a Cause, of Motor Neuron Dysfunction
Rocky G. Gogliotti et al.
JOURNAL OF NEUROSCIENCE (2012)
Interdigitated array of Pt electrodes for electrical stimulation and engineering of aligned muscle tissue
Samad Ahadian et al.
LAB ON A CHIP (2012)
Myoblasts Derived From Normal hESCs and Dystrophic hiPSCs Efficiently Fuse With Existing Muscle Fibers Following Transplantation
Sebastien Goudenege et al.
MOLECULAR THERAPY (2012)
Functional Neuromuscular Junctions Formed by Embryonic Stem Cell-Derived Motor Neurons
Joy A. Umbach et al.
PLOS ONE (2012)
Drug Screening for ALS Using Patient-Specific Induced Pluripotent Stem Cells
Naohiro Egawa et al.
SCIENCE TRANSLATIONAL MEDICINE (2012)
Molecular, Physiological, and Motor Performance Defects in DMSXL Mice Carrying >1,000 CTG Repeats from the Human DM1 Locus
Aline Huguet et al.
PLOS GENETICS (2012)
Human Neural Crest Stem Cells Derived from Human ESCs and Induced Pluripotent Stem Cells: Induction, Maintenance, and Differentiation into Functional Schwann Cells
Qiuyue Liu et al.
STEM CELLS TRANSLATIONAL MEDICINE (2012)
Myasthenia Gravis: A Review
Annapurni Jayam Trouth et al.
AUTOIMMUNE DISEASES (2012)
Alternative splicing regulation by Muscleblind proteins: from development to disease
Juan M. Fernandez-Costa et al.
BIOLOGICAL REVIEWS (2011)
Neuromuscular junction formation between human stem cell-derived motoneurons and human skeletal muscle in a defined system
Xiufang Guo et al.
BIOMATERIALS (2011)
The role of extracellular matrix composition in structure and function of bioengineered skeletal muscle
Sara Hinds et al.
BIOMATERIALS (2011)
Neuron-glia interactions: the roles of Schwann cells in neuromuscular synapse formation and function
Yoshie Sugiura et al.
BIOSCIENCE REPORTS (2011)
Mutant Human Embryonic Stem Cells Reveal Neurite and Synapse Formation Defects in Type 1 Myotonic Dystrophy
Antoine Marteyn et al.
CELL STEM CELL (2011)
Conversion of Mouse and Human Fibroblasts into Functional Spinal Motor Neurons
Esther Y. Son et al.
CELL STEM CELL (2011)
The clinical and electrodiagnostic characteristics of Pompe disease with post-enzyme replacement therapy findings
Lisa D. Hobson-Webb et al.
CLINICAL NEUROPHYSIOLOGY (2011)
Hind limb muscle atrophy precedes cerebral neuronal degeneration in G93A-SOD1 mouse model of amyotrophic lateral sclerosis: A longitudinal MRI study
Stefania Marcuzzo et al.
EXPERIMENTAL NEUROLOGY (2011)
Peripheral Neuropathy Is Linked to a Severe Form of Myotonic Dystrophy in Transgenic Mice
Petrica-Adrian Panaite et al.
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (2011)
Schwann Cell Mitochondrial Metabolism Supports Long-Term Axonal Survival and Peripheral Nerve Function
Andreu Viader et al.
JOURNAL OF NEUROSCIENCE (2011)
Rapid and Efficient Generation of Functional Motor Neurons From Human Pluripotent Stem Cells Using Gene Delivered Transcription Factor Codes
Mark E. Hester et al.
MOLECULAR THERAPY (2011)
SOD1 and TDP-43 animal models of amyotrophic lateral sclerosis: recent advances in understanding disease toward the development of clinical treatments
Peter I. Joyce et al.
MAMMALIAN GENOME (2011)
Tbx6-dependent Sox2 regulation determines neural or mesodermal fate in axial stem cells
Tatsuya Takemoto et al.
NATURE (2011)
Misregulated alternative splicing of BIN1 is associated with T tubule alterations and muscle weakness in myotonic dystrophy
Charlotte Fugier et al.
NATURE MEDICINE (2011)
HDAC1 and HDAC2 control the transcriptional program of myelination and the survival of Schwann cells
Claire Jacob et al.
NATURE NEUROSCIENCE (2011)
Altered Expression of Myogenic Regulatory Factors in the Mouse Model of Amyotrophic Lateral Sclerosis
Raquel Manzano et al.
NEURODEGENERATIVE DISEASES (2011)
SPP1 GENOTYPE IS A DETERMINANT OF DISEASE SEVERITY IN DUCHENNE MUSCULAR DYSTROPHY PREDICTING THE SEVERITY OF DUCHENNE MUSCULAR DYSTROPHY: IMPLICATIONS FOR TREATMENT
Theodoros Kyriakides
NEUROLOGY (2011)
Hypoglossal neuropathology and respiratory activity in Pompe mice
Kun-Ze Lee et al.
FRONTIERS IN PHYSIOLOGY (2011)
A defined long-term in vitro tissue engineered model of neuromuscular junctions
Mainak Das et al.
BIOMATERIALS (2010)
Short Telomeres and Stem Cell Exhaustion Model Duchenne Muscular Dystrophy in mdx/mTR Mice
Alessandra Sacco et al.
CELL (2010)
Highly Efficient Reprogramming to Pluripotency and Directed Differentiation of Human Cells with Synthetic Modified mRNA
Luigi Warren et al.
CELL STEM CELL (2010)
Retrograde influence of muscle fibers on their innervation revealed by a novel marker for slow motoneurons
Joe V. Chakkalakal et al.
DEVELOPMENT (2010)
Oxidative stress in ALS: Key role in motor neuron injury and therapeutic target
Sian C. Barber et al.
FREE RADICAL BIOLOGY AND MEDICINE (2010)
Inducible Lineage Tracing of Pax7-Descendant Cells Reveals Embryonic Origin of Adult Satellite Cells
Christoph Lepper et al.
GENESIS (2010)
Identification of Novel Spinal Cholinergic Genetic Subtypes Disclose Chodl and Pitx2 as Markers for Fast Motor Neurons and Partition Cells
Anders Enjin et al.
JOURNAL OF COMPARATIVE NEUROLOGY (2010)
Neuronal NOS is dislocated during muscle atrophy in amyotrophic lateral sclerosis
Naoki Suzuki et al.
JOURNAL OF THE NEUROLOGICAL SCIENCES (2010)
The evidence for altered RNA metabolism in amyotrophic lateral sclerosis (ALS)
Michael J. Strong
JOURNAL OF THE NEUROLOGICAL SCIENCES (2010)
Differences in the predominance of lysosomal and autophagic pathologies between infants and adults with Pompe disease: implications for therapy
Nina Raben et al.
MOLECULAR GENETICS AND METABOLISM (2010)
Cross-reactive immunologic material status affects treatment outcomes in Pompe disease infants
Priya S. Kishnani et al.
MOLECULAR GENETICS AND METABOLISM (2010)
Progressive skeletal muscle weakness in transgenic mice expressing CTG expansions is associated with the activation of the ubiquitin-proteasome pathway
Alban Vignaud et al.
NEUROMUSCULAR DISORDERS (2010)
A Randomized Study of Alglucosidase Alfa in Late-Onset Pompe's Disease.
Ans T. van der Ploeg et al.
NEW ENGLAND JOURNAL OF MEDICINE (2010)
Deficits in axonal transport precede ALS symptoms in vivo
Lynsey G. Bilsland et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2010)
Nrg1/ErbB signaling networks in Schwann cell development and myelination
Jason Newbern et al.
SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY (2010)
Inhibition of Notch Signaling in Human Embryonic Stem Cell-Derived Neural Stem Cells Delays G1/S Phase Transition and Accelerates Neuronal Differentiation In Vitro and In Vivo
Lodovica Borghese et al.
STEM CELLS (2010)
C. ELEGANS MODELS OF NEUROMUSCULAR DISEASES EXPEDITE TRANSLATIONAL RESEARCH
James N. Sleigh et al.
TRANSLATIONAL NEUROSCIENCE (2010)
Influence of Disease Progression on the Neuromuscular Blocking Effect of Mivacurium in Children and Adolescents with Duchenne Muscular Dystrophy
Harald Ihmsen et al.
ANESTHESIOLOGY (2009)
The neurobiology of the dystrophin-associated glycoprotein complex
Adrian Waite et al.
ANNALS OF MEDICINE (2009)
Pathogenic mechanisms of myotonic dystrophy
Johanna E. Lee et al.
BIOCHEMICAL SOCIETY TRANSACTIONS (2009)
Microsatellite repeat instability and neurological disease
Judith R. Brouwer et al.
BIOESSAYS (2009)
Redefining the Progression of Lineage Segregations during Mammalian Embryogenesis by Clonal Analysis
Elena Tzouanacou et al.
DEVELOPMENTAL CELL (2009)
Latent TGF-β-binding protein 4 modifies muscular dystrophy in mice
Ahlke Heydemann et al.
JOURNAL OF CLINICAL INVESTIGATION (2009)
Uniquely Hominid Features of Adult Human Astrocytes
Nancy Ann Oberheim et al.
JOURNAL OF NEUROSCIENCE (2009)
Highly efficient neural conversion of human ES and iPS cells by dual inhibition of SMAD signaling
Stuart M. Chambers et al.
NATURE BIOTECHNOLOGY (2009)
Notch controls embryonic Schwann cell differentiation, postnatal myelination and adult plasticity
Ashwin Woodhoo et al.
NATURE NEUROSCIENCE (2009)
Differentiation of spinal motor neurons from pluripotent human stem cells
Bao-Yang Hu et al.
NATURE PROTOCOLS (2009)
Retinoid Signaling and Neurogenin2 Function Are Coupled for the Specification of Spinal Motor Neurons through a Chromatin Modifier CBP
Seunghee Lee et al.
NEURON (2009)
Early Treatment With Alglucosidase Alfa Prolongs Long-Term Survival of Infants With Pompe Disease
Priya S. Kishnani et al.
PEDIATRIC RESEARCH (2009)
Neural deficits contribute to respiratory insufficiency in Pompe disease
Lara R. DeRuisseau et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)
Regulation of Neural Specification from Human Embryonic Stem Cells by BMP and FGF
Timothy M. LaVaute et al.
STEM CELLS (2009)
ALSOD: The Amyotrophic Lateral Sclerosis Online Database
Richard Wroe et al.
AMYOTROPHIC LATERAL SCLEROSIS (2008)
Presymptomatic biochemical changes in hindlimb muscle of G93A human Cu/Zn superoxide dismutase 1 transgenic mouse model of amyotrophic lateral sclerosis
Kevin H. J. Park et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE (2008)
Localized acetylcholine receptor clustering dynamics in response to microfluidic focal stimulation with agrin
Anna Tourovskaia et al.
BIOPHYSICAL JOURNAL (2008)
Skeletal Muscle Is a Primary Target of SOD1G93A-Mediated Toxicity
Gabriella Dobrowolny et al.
CELL METABOLISM (2008)
Development of the Schwann Cell Lineage: From the Neural Crest to the Myelinated Nerve
Ashwin Woodhoo et al.
GLIA (2008)
Identity, developmental restriction and reactivity of extralaminar cells capping mammalian neuromuscular junctions
Felipe A. Court et al.
JOURNAL OF CELL SCIENCE (2008)
Animal models for genetic neuromuscular diseases
Mariz Vainzof et al.
JOURNAL OF MOLECULAR NEUROSCIENCE (2008)
Myotonic dystrophy transgenic mice exhibit pathologic abnormalities in diaphragm neuromuscular junctions and phrenic nerves
Petrica-Adrian Panaite et al.
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (2008)
Myelination in mouse dorsal root ganglion/Schwann cell cocultures
Satu Paeivaelaeinen et al.
MOLECULAR AND CELLULAR NEUROSCIENCE (2008)
Direct Muscle Delivery of GDNF With Human Mesenchymal Stem Cells Improves Motor Neuron Survival and Function in a Rat Model of Familial ALS
Masatoshi Suzuki et al.
MOLECULAR THERAPY (2008)
Systemic microdystrophin gene delivery improves skeletal muscle structure and function in old dystrophic mdx mice
Paul Gregorevic et al.
MOLECULAR THERAPY (2008)
In vitro study of axonal migration and myelination of motor neurons in a three-dimensional tissue-engineered model
Marie Gingras et al.
GLIA (2008)
MuSK controls where motor axons grow and form synapses
Natalie Kim et al.
NATURE NEUROSCIENCE (2008)
MBNL binds similar RNA structures in the CUG repeats of myotonic dystrophy and its pre-mRNA substrate cardiac troponin T
M. Bryan Warf et al.
RNA (2007)
Time course of preferential motor unit loss in the SODIG93A mouse model of amyotrophic lateral sclerosis
J. Hegedus et al.
NEUROBIOLOGY OF DISEASE (2007)
Activation of the Nrf2-ARE pathway in muscle and spinal cord during ALS-like pathology in mice expressing mutant SOD1
Andrew D. Kraft et al.
EXPERIMENTAL NEUROLOGY (2007)
GDNF Secreting Human Neural Progenitor Cells Protect Dying Motor Neurons, but Not Their Projection to Muscle, in a Rat Model of Familial ALS
Masatoshi Suzuki et al.
PLOS ONE (2007)
Altered mRNA splicing of dystrophin in type 1 myotonic dystrophy
Masayuki Nakamori et al.
MUSCLE & NERVE (2007)
Isolation, purification and expansion of myelination-competent, neonatal mouse Schwann cells
Henrika Honkanen et al.
EUROPEAN JOURNAL OF NEUROSCIENCE (2007)
Differential muscular glycogen clearance after enzyme replacement therapy in a mouse model of Pompe disease
Michael L. Hawes et al.
MOLECULAR GENETICS AND METABOLISM (2007)
Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons
Makiko Nagai et al.
NATURE NEUROSCIENCE (2007)
Interplay of IKK/NF-κB signaling in macrophages and myofibers promotes muscle degeneration in Duchenne muscular dystrophy
Swarnali Acharyya et al.
JOURNAL OF CLINICAL INVESTIGATION (2007)
Functional neural development from human embryonic stem cells: Accelerated synaptic activity via astrocyte coculture
M. Austin Johnson et al.
JOURNAL OF NEUROSCIENCE (2007)
NMDA receptor subunits have differential roles in mediating excitotoxic neuronal death both in vitro and in vivo
Yitao Liu et al.
JOURNAL OF NEUROSCIENCE (2007)
Ribonuclear foci at the neuromuscular junction in myotonic dystrophy type 1
T. M. Wheeler et al.
NEUROMUSCULAR DISORDERS (2007)
The Notch response inhibitor DAPT enhances neuronal differentiation in embryonic stem cell-derived embryoid bodies independently of Sonic Hedgehog signaling
T. Quinn Crawford et al.
DEVELOPMENTAL DYNAMICS (2007)
Muscle-derived but not centrally derived transgene GDNF is neuroprotective in G93A-SOD1 mouse model of ALS
Wen Li et al.
EXPERIMENTAL NEUROLOGY (2007)
Acid alpha-glucosidase deficiency (Pompe disease)
Tokiko Fukuda et al.
CURRENT NEUROLOGY AND NEUROSCIENCE REPORTS (2007)
Distal excitability changes in motor axons in amyotrophic lateral sclerosis
Miho Nakata et al.
CLINICAL NEUROPHYSIOLOGY (2006)
Inhibition of glycogen synthase kinase-3β activity is sufficient to stimulate myogenic differentiation
JLJ van der Velden et al.
AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY (2006)
Systemic administration of L-arginine benefits mdx skeletal muscle function
ER Barton et al.
MUSCLE & NERVE (2005)
Terminal schwann cell structure is altered in diaphragm of mdx mice
KE Personius et al.
MUSCLE & NERVE (2005)
Neuromuscular synapses can form in vivo by incorporation of initially aneural postsynaptic specializations
H Flanagan-Steet et al.
DEVELOPMENT (2005)
Regulation of Tbx6 expression by Notch signaling
PH White et al.
GENESIS (2005)
Rat embryonic motoneurons in long-term co-culture with Schwann cells - a system to investigate motoneuron diseases on a cellular level in vitro
K Haastert et al.
JOURNAL OF NEUROSCIENCE METHODS (2005)
Syne proteins anchor muscle nuclei at the neuromuscular junction
RM Grady et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2005)
Directed differentiation of telencephalic precursors from embryonic stem cells
K Watanabe et al.
NATURE NEUROSCIENCE (2005)
Specification of motoneurons from human embryonic stem cells
XJ Li et al.
NATURE BIOTECHNOLOGY (2005)
Distinct effects of caudalizing factors on regional specification of embryonic stem cell-derived neural precursors
T Irioka et al.
DEVELOPMENTAL BRAIN RESEARCH (2005)
The origin and development of glial cells in peripheral nerves
KR Jessen et al.
NATURE REVIEWS NEUROSCIENCE (2005)
Opposing FGF and retinoid pathways: a signalling switch that controls differentiation and patterning onset in the extending vertebrate body axis
R Diez del Corral et al.
BIOESSAYS (2004)
Protein aggregation and neurodegenerative disease
CA Ross et al.
NATURE MEDICINE (2004)
Cellular and molecular regulation of muscle regeneration
SBP Charge et al.
PHYSIOLOGICAL REVIEWS (2004)
Glial cells maintain synaptic structure and function and promote development of the neuromuscular junction in vivo
LV Reddy et al.
NEURON (2003)
The concerted action of Meox homeobox genes is required upstream of genetic pathways essential for the formation, patterning and differentiation of somites
BS Mankoo et al.
DEVELOPMENT (2003)
Histopathological differences of myotonic dystrophy type 1 (DM1) and PROMM/DM2
A Vihola et al.
NEUROLOGY (2003)
Wnt3A plays a major role in the segmentation clock controlling somitogenesis
A Aulehla et al.
DEVELOPMENTAL CELL (2003)
Hyperproliferation of synapses on spinal motor neurons of Duchenne muscular dystrophy and myotonic dystrophy patients
M Nagao et al.
ACTA NEUROPATHOLOGICA (2003)
Mechanical stimulation improves tissue-engineered human skeletal muscle
CA Powell et al.
AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY (2002)
Directed differentiation of embryonic stem cells into motor neurons
H Wichterle et al.
CELL (2002)
Accumulation of SOD1 mutants in postnatal motoneurons does not cause motoneuron pathology or motoneuron disease
MM Lino et al.
JOURNAL OF NEUROSCIENCE (2002)
Three proteins, MBNL, MBLL and MBXL, co-localize in vivo with nuclear foci of expanded-repeat transcripts in DM1 and DM2 cells
M Fardaei et al.
HUMAN MOLECULAR GENETICS (2002)
Assigning the positional identity of spinal motor neurons: Rostrocaudal patterning of Hox-c expression by FGFs, Gdf11, and retinoids
JP Liu et al.
NEURON (2001)
Schwann cells express active agrin and enhance aggregation of acetylcholine receptors on muscle fibers
JF Yang et al.
JOURNAL OF NEUROSCIENCE (2001)
Conditional tissue-specific expression of the acid α-glucosidase (GAA) gene in the GAA knockout mice:: implications for therapy
N Raben et al.
HUMAN MOLECULAR GENETICS (2001)
The murine winged helix transcription factors, Foxc1 and Foxc2, are both required for cardiovascular development and somitogenesis
T Kume et al.
GENES & DEVELOPMENT (2001)
Intractable fever and cortical neuronal glycogen storage in glycogenosis type 2
C Martini et al.
NEUROLOGY (2001)
The muscle-specific enolase is an early marker of human myogenesis
F Fougerousse et al.
JOURNAL OF MUSCLE RESEARCH AND CELL MOTILITY (2001)
FGF signaling controls somite boundary position and regulates segmentation clock control of spatiotemporal Hox gene activation
J Dubrulle et al.
CELL (2001)
FGF signaling regulates mesoderm cell fate specification and morphogenetic movement at the primitive streak
B Ciruna et al.
DEVELOPMENTAL CELL (2001)
Distinct roles of nerve and muscle in postsynaptic differentiation of the neuromuscular synapse
WC Lin et al.
NATURE (2001)
The acquisition of motoneuron subtype identity and motor circuit formation
LT Landmesser
INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE (2001)
The dystroglycan complex is necessary for stabilization of acetylcholine receptor clusters at neuromuscular junctions and formation of the synaptic basement membrane
C Jacobson et al.
JOURNAL OF CELL BIOLOGY (2001)
The retinoic acid-inactivating enzyme CYP26 is essential for establishing an uneven distribution of retinoic acid along the anterio-posterior axis within the mouse embryo
Y Sakai et al.
GENES & DEVELOPMENT (2001)
Neuronal specification in the spinal cord: Inductive signals and transcriptional codes
TM Jessell
NATURE REVIEWS GENETICS (2000)
Severe anaesthetic incidents in patients and families with Duchenne and Becker type muscular dystrophy
E Breucking et al.
ANAESTHESIST (2000)
Aberrant development of motor axons and neuromuscular synapses in erbB2-deficient mice
WC Lin et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2000)
A mouse model for spinal muscular atrophy
HM Hsieh-Li et al.
NATURE GENETICS (2000)
Share Paper
