The DOI matches its Crossref record; 45 of the 45 references it cites were found in a registry or an index.
Time course of preferential motor unit loss in the SOD1G93A mouse model of amyotrophic lateral sclerosis
Open accessAbstract
Electromyographical analyses of pre-symptomatic motor unit loss in the SOD1 G93A transgenic mouse model of amyotrophic lateral sclerosis (ALS) have yielded contradictory findings as to the onset and time course. We recorded hindlimb muscle and motor unit isometric forces to determine motor unit number and size throughout the life span of the mice. Motor unit numbers in fast-twitch tibialis anterior, extensor digitorum longus and medial gastrocnemius muscles declined from 40 days of age, 50 days before reported overt symptoms and motoneuron loss. Motor unit numbers fell after overt symptoms in the slow-twitch soleus muscle. Muscle forces declined in parallel with motor unit numbers, indicating little or no functional compensation by sprouting. Early muscle-specific decline was due to selective preferential vulnerability of large, fast motor units, innervated by large motoneurons. Large motoneurons are hence the most vulnerable in ALS with die-back occurring prior to overt symptoms. We conclude that size of motoneurons, their axons, and their motor unit size are important determinants of motoneuron susceptibility in ALS.
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Motor neuron vulnerability and resistance in amyotrophic lateral sclerosis
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Neuronal Matrix Metalloproteinase-9 Is a Determinant of Selective Neurodegeneration
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Metadata
- Authors
- Hegedus, J., Putman, C. T., & Gordon, T.
- Venue
- Neurobiology of Disease
- Publisher
- Elsevier BV
- Published
- 2007-11
- Volume
- 28
- Issue
- 2
- Pages
- 154-164
- ISSN
- 0969-9961 (ISSN-L)
- OpenAlex
- W2027886291
- Cited by
- 321
- References
- 45
- FWCI
- 6.76
- Type
- journal article
- Language
- en
- Open access
- green
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- Hegedus, J., Putman, C. T., & Gordon, T. (2007). Time course of preferential motor unit loss in the SOD1G93A mouse model of amyotrophic lateral sclerosis. Neurobiology of Disease. https://doi.org/10.1016/j.nbd.2007.07.003
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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