ArticleProceedings of the National Academy of Sciences of the United States of America2014
Selective degeneration of a physiological subtype of spinal motor neuron in mice with SOD1-linked ALS.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers.
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Who cites it
37 citing papers in PubMed.
- Metabolic alterations in the absence of a neuromuscular phenotype in humanizedBrain communications · 2026Article
- Brain Connectivity, Neural Networks, and Resilience in Aging and Neurodegeneration.The American journal of pathology · 2025Review
- Effect of size on expression of bistability in mouse spinal motoneurons.Journal of neurophysiology · 2024Article
- Article
- Motoneuron excitability dysfunction in ALS: Pseudo-mystery or authentic conundrum?The Journal of physiology · 2022Review
- In vitro longitudinal lumbar spinal cord preparations to study sensory and recurrent motor microcircuits of juvenile mice.Journal of neurophysiology · 2022Article
- Singling out motor neurons in the age of single-cell transcriptomics.Trends in genetics : TIG · 2022Review
- Slow motor neurons resist pathological TDP-43 and mediate motor recovery in the rNLS8 model of amyotrophic lateral sclerosis.Acta neuropathologica communications · 2022Article
- Identification of adult spinal Shox2 neuronal subpopulations based on unbiased computational clustering of electrophysiological properties.Frontiers in neural circuits · 2022Article
- Electrical and Morphological Properties of Developing Motoneurons in Postnatal Mice and Early Abnormalities in SOD1 Transgenic Mice.Advances in neurobiology · 2022Review
- Characterization of laryngeal motor neuron properties in the American bullfrog, Lithobates catesbieanus.Respiratory physiology & neurobiology · 2021Article
- Locomotor deficits in a mouse model of ALS are paralleled by loss of V1-interneuron connections onto fast motor neurons.Nature communications · 2021Article
- Single nucleus RNA-sequencing defines unexpected diversity of cholinergic neuron types in the adult mouse spinal cord.Nature communications · 2021Article
- Estimating the effects of slicing on the electrophysiological properties of spinal motoneurons under normal and disease conditions.Journal of neurophysiology · 2021Article
- Chronic defects in intraspinal mechanisms of spike encoding by spinal motoneurons following chemotherapy.Experimental neurology · 2020Article
- Molecular and electrophysiological properties of mouse motoneuron and motor unit subtypes.Current opinion in physiology · 2019Article
- Ion channel dysfunction and altered motoneuron excitability in ALS.Neurological disorders & epilepsy journal · 2019Article
- Motor Neuron Susceptibility in ALS/FTD.Frontiers in neuroscience · 2019Review
- Repurposing carbamazepine for the treatment of amyotrophic lateral sclerosis in SOD1-G93A mouse model.CNS neuroscience & therapeutics · 2018Article
- Kir4.1-Dependent Astrocyte-Fast Motor Neuron Interactions Are Required for Peak Strength.Neuron · 2018Article
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6 authors.
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Abstract
Amyotrophic lateral sclerosis (ALS; Lou Gehrig's disease) affects motor neurons (MNs) in the brain and spinal cord. Understanding the pathophysiology of this condition seems crucial for therapeutic design, yet few electrophysiological studies in actively degenerating animal models have been reported. Here, we report a novel preparation of acute slices from adult mouse spinal cord, allowing visualized whole cell patch-clamp recordings of fluorescent lumbar MN cell bodies from ChAT-eGFP or superoxide dismutase 1-yellow fluorescent protein (SOD1YFP) transgenic animals up to 6 mo of age. We examined 11 intrinsic electrophysiologic properties of adult ChAT-eGFP mouse MNs and classified them into four subtypes based on these parameters. The subtypes could be principally correlated with instantaneous (initial) and steady-state firing rates. We used retrograde tracing using fluorescent dye injected into fast or slow twitch lower extremity muscle with slice recordings from the fluorescent-labeled lumbar MN cell bodies to establish that fast and slow firing MNs are connected with fast and slow twitch muscle, respectively. In a G85R SOD1YFP transgenic mouse model of ALS, which becomes paralyzed by 5-6 mo, where MN cell bodies are fluorescent, enabling the same type of recording from spinal cord tissue slices, we observed that all four MN subtypes were present at 2 mo of age. At 4 mo, by which time substantial neuronal SOD1YFP aggregation and cell loss has occurred and symptoms have developed, one of the fast firing subtypes that innvervates fast twitch muscle was lost. These results begin to describe an order of the pathophysiologic events in ALS.
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