| Excitability properties of mouse motor axons in the mutant SOD1(G93A) model of amyotrophic lateral sclerosis. | |
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MedLine Citation:
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PMID: 20095022 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Non-invasive excitability studies of motor axons in patients with amyotrophic lateral sclerosis (ALS) have revealed a changing pattern of abnormal membrane properties with disease progression, but the heterogeneity of the changes has made it difficult to relate them to pathophysiology. The SOD1(G93A) mouse model of ALS displays more synchronous motoneuron pathology. Multiple excitability measures of caudal and sciatic nerves in mutant and wild-type mice were compared before onset of signs and during disease progression (4-19 weeks), and they were related to changes in muscle fiber histochemistry. Excitability differences indicated a modest membrane depolarization in SOD1(G93A) axons at about the time of symptom onset (8 weeks), possibly due to deficient energy supply. Previously described excitability changes in ALS patients, suggesting altered sodium and potassium conductances, were not seen in the mice. This suggests that those changes relate to features of the human disease that are not well represented in the animal model. |
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Authors:
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Delphine Boërio; Bernadett Kalmar; Linda Greensmith; Hugh Bostock |
Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Muscle & nerve Volume: 41 ISSN: 1097-4598 ISO Abbreviation: Muscle Nerve Publication Date: 2010 Jun |
Date Detail:
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Created Date: 2010-05-31 Completed Date: 2010-07-07 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7803146 Medline TA: Muscle Nerve Country: United States |
Other Details:
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Languages: eng Pagination: 774-84 Citation Subset: IM |
Affiliation:
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Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, Queen Square, London WC1N 3BG, UK. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Amyotrophic Lateral Sclerosis
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enzymology,
genetics* Animals Axons / pathology*, physiology Disease Models, Animal Electric Stimulation Humans Mice Motor Neurons / pathology*, physiology Mutation Peripheral Nerves / pathology, physiopathology Polymorphism, Single Nucleotide* Superoxide Dismutase / genetics* Tail / innervation Tibial Nerve / pathology, physiopathology |
| Chemical | |
Reg. No./Substance:
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EC 1.15.1.1/Superoxide Dismutase |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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