ReviewFrontiers in human neuroscience2014
Motoneuron firing in amyotrophic lateral sclerosis (ALS).
Review in Frontiers in human neuroscience, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed.
- A Xerotic Skin-Compatible Neuromotor Electrode Interface for Non-Invasive Muscle Weakness Evaluation in Dermatomyositis.Advanced materials (Deerfield Beach, Fla.) · 2026Article
- Machine-learning classification of motor unit types in the adult mouse.The Journal of physiology · 2026Article
- Translation of surface electromyography into a clinically applicable objective bulbar assessment tool to improve measurement-based care in amyotrophic laterals sclerosis.Frontiers in neuroscience · 2026Article
- Article
- Machine-Learning Classification of Motor Unit Types in the Adult Mouse.bioRxiv : the preprint server for biology · 2025Article
- A multimodal approach to automated hierarchical assessment of bulbar involvement in amyotrophic lateral sclerosis.Frontiers in neurology · 2024Article
- Selective Muscle Involvement in Amyotrophic Lateral Sclerosis: Evidence Inferred from the Point of Motor Unit Firing Rates.Noro psikiyatri arsivi · 2024Article
- A novel muscle network approach for objective assessment and profiling of bulbar involvement in ALS.Frontiers in neuroscience · 2024Article
- RNA-binding proteins as a common ground for neurodegeneration and inflammation in amyotrophic lateral sclerosis and multiple sclerosis.Frontiers in molecular neuroscience · 2023Review
- Shaky hands are a part of motor neuron disease phenotype: clinical and electrophysiological study of 77 patients.Journal of neurology · 2022Article
- The strength of corticomotoneuronal drive underlies ALS split phenotypes and reflects early upper motor neuron dysfunction.Brain and behavior · 2021Review
- Proteostatic imbalance and protein spreading in amyotrophic lateral sclerosis.The EMBO journal · 2021Review
- Combination of acamprosate and baclofen (PXT864) as a potential new therapy for amyotrophic lateral sclerosis.Journal of neuroscience research · 2020Article
- Neurophysiological Characterization of a Non-Human Primate Model of Traumatic Spinal Cord Injury Utilizing Fine-Wire EMG Electrodes.Sensors (Basel, Switzerland) · 2019Article
- In vivo evidence for reduced ion channel expression in motor axons of patients with amyotrophic lateral sclerosis.The Journal of physiology · 2018Article
- Chronic electromyograms in treadmill running SOD1 mice reveal early changes in muscle activation.The Journal of physiology · 2017Article
- New perspectives on amyotrophic lateral sclerosis: the role of glial cells at the neuromuscular junction.The Journal of physiology · 2017Review
- Adducin at the Neuromuscular Junction in Amyotrophic Lateral Sclerosis: Hanging on for Dear Life.Frontiers in cellular neuroscience · 2016Review
- Mechanisms underlying firing in healthy and sick human motoneurons.Frontiers in human neuroscience · 2015Article
- Valproate Attenuates 25-kDa C-Terminal Fragment of TDP-43-Induced Neuronal Toxicity via Suppressing Endoplasmic Reticulum Stress and Activating Autophagy.International journal of biological sciences · 2015Article
Corrections and comments
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Authors and funding
4 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Amyotrophic lateral sclerosis is an inexorably progressive neurodegenerative disorder involving the classical motor system and the frontal effector brain, causing muscular weakness and atrophy, with variable upper motor neuron signs and often an associated fronto-temporal dementia. The physiological disturbance consequent on the motor system degeneration is beginning to be well understood. In this review we describe aspects of the motor cortical, neuronal, and lower motor neuron dysfunction. We show how studies of the changes in the pattern of motor unit firing help delineate the underlying pathophysiological disturbance as the disease progresses. Such studies are beginning to illuminate the underlying disordered pathophysiological processes in the disease, and are important in designing new approaches to therapy and especially for clinical trials.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.