ArticleProceedings of the National Academy of Sciences of the United States of America2016
Reduced high-frequency motor neuron firing, EMG fractionation, and gait variability in awake walking ALS mice.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
What it found
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Who cites it
20 citing papers in PubMed, 35 citations in OpenAlex.
- Article
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- CORP: In vivo muscle strength-perspectives on the design and interpretation of preclinical animal studies.American journal of physiology. Regulatory, integrative and comparative physiology · 2025Review
- Motor unit mechanisms of speed control in mouse locomotion.bioRxiv : the preprint server for biology · 2025Article
- X-Ray Fluoroscopy-Based Kinematic Analysis of Quadrupedal Locomotion in Slow and Fast Fatigue-Resistant Motor Neuron-Deleted Mice.Muscle & nerve · 2025Article
- Alterations in neuromuscular junction morphology with ageing and endurance training modulate neuromuscular transmission and myofibre composition.The Journal of physiology · 2025Article
- All-in-one wearable drug efficacy assessment systems for bulbar muscle function using amyotrophic lateral sclerosis animal models.Nature communications · 2024Article
- Ultraflexible electrodes for recording neural activity in the mouse spinal cord during motor behavior.Cell reports · 2024Article
- A signal EMerGes from the noise.Cell reports methods · 2023Article
- Striatal CDK5 Regulates Cholinergic Neuron Activation and Dyskinesia-like Behaviors through BK Channels.Research (Washington, D.C.) · 2023Article
- Aging-Dependent Altered Transcriptional Programs Underlie Activity Impairments in Human C9orf72-Mutant Motor Neurons.Frontiers in molecular neuroscience · 2022Article
- From Physiological Properties to Selective Vulnerability of Motor Units in Amyotrophic Lateral Sclerosis.Advances in neurobiology · 2022Article
- Real-Time Functional Assay of Volumetric Muscle Loss Injured Mouse Masseter Muscles via Nanomembrane Electronics.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2021Article
- Neuromodulation of the cerebellum rescues movement in a mouse model of ataxia.Nature communications · 2021Article
- Article
- Locally coupled electromechanical interfaces based on cytoadhesion-inspired hybrids to identify muscular excitation-contraction signatures.Nature communications · 2020Article
- Imaging spinal cord activity in behaving animals.Experimental neurology · 2019Review
- The Subprimary Range of Firing Is Present in Both Cat and Mouse Spinal Motoneurons and Its Relationship to Force Development Is Similar for the Two Species.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2018Article
- Opportunities and Challenges for Single-Unit Recordings from Enteric Neurons in Awake Animals.Micromachines · 2018Review
- Article
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Authors and funding
9 authors at 3 institutions in 1 country.
Funding
Abstract
Amyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative disease prominently featuring motor neuron (MN) loss and paralysis. A recent study using whole-cell patch clamp recording of MNs in acute spinal cord slices from symptomatic adult ALS mice showed that the fastest firing MNs are preferentially lost. To measure the in vivo effects of such loss, awake symptomatic-stage ALS mice performing self-initiated walking on a wheel were studied. Both single-unit extracellular recordings within spinal cord MN pools for lower leg flexor and extensor muscles and the electromyograms (EMGs) of the corresponding muscles were recorded. In the ALS mice, we observed absent or truncated high-frequency firing of MNs at the appropriate time in the step cycle and step-to-step variability of the EMG, as well as flexor-extensor coactivation. In turn, kinematic analysis of walking showed step-to-step variability of gait. At the MN level, the higher frequencies absent from recordings from mutant mice corresponded with the upper range of frequencies observed for fast-firing MNs in earlier slice measurements. These results suggest that, in SOD1-linked ALS mice, symptoms are a product of abnormal MN firing due at least in part to loss of neurons that fire at high frequency, associated with altered EMG patterns and hindlimb kinematics during gait.
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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.