Motoneuronal Spinal Circuits in Degenerative Motoneuron Disease
The most evident phenotype of degenerative motoneuron disease is the loss of motor function which accompanies motoneuron death. In both amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA), it is now clear that dysfunction is not restricted to motoneurons but is manifest in the spin...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2020-05-01
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| Series: | Frontiers in Molecular Neuroscience |
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| Online Access: | https://www.frontiersin.org/article/10.3389/fnmol.2020.00074/full |
| _version_ | 1819019885703856128 |
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| author | Mélanie Falgairolle Michael J. O’Donovan |
| author_facet | Mélanie Falgairolle Michael J. O’Donovan |
| author_sort | Mélanie Falgairolle |
| collection | DOAJ |
| description | The most evident phenotype of degenerative motoneuron disease is the loss of motor function which accompanies motoneuron death. In both amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA), it is now clear that dysfunction is not restricted to motoneurons but is manifest in the spinal circuits in which motoneurons are embedded. As mounting evidence shows that motoneurons possess more elaborate and extensive connections within the spinal cord than previously realized, it is necessary to consider the role of this circuitry and its dysfunction in the disease process. In this review article, we ask if the selective vulnerability of the different motoneuron types and the relative disease resistance of distinct motoneuron groups can be understood in terms of their intraspinal connections. |
| first_indexed | 2024-12-21T03:42:26Z |
| format | Article |
| id | doaj.art-f08f3c648cc3415fa3b91af978d33aae |
| institution | Directory Open Access Journal |
| issn | 1662-5099 |
| language | English |
| last_indexed | 2024-12-21T03:42:26Z |
| publishDate | 2020-05-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Molecular Neuroscience |
| spelling | doaj.art-f08f3c648cc3415fa3b91af978d33aae2022-12-21T19:17:11ZengFrontiers Media S.A.Frontiers in Molecular Neuroscience1662-50992020-05-011310.3389/fnmol.2020.00074537045Motoneuronal Spinal Circuits in Degenerative Motoneuron DiseaseMélanie FalgairolleMichael J. O’DonovanThe most evident phenotype of degenerative motoneuron disease is the loss of motor function which accompanies motoneuron death. In both amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA), it is now clear that dysfunction is not restricted to motoneurons but is manifest in the spinal circuits in which motoneurons are embedded. As mounting evidence shows that motoneurons possess more elaborate and extensive connections within the spinal cord than previously realized, it is necessary to consider the role of this circuitry and its dysfunction in the disease process. In this review article, we ask if the selective vulnerability of the different motoneuron types and the relative disease resistance of distinct motoneuron groups can be understood in terms of their intraspinal connections.https://www.frontiersin.org/article/10.3389/fnmol.2020.00074/fulllocomotionspinal muscular atrophyamyotrophic lateral sclerosiscentral pattern generatorrecurrent collaterals |
| spellingShingle | Mélanie Falgairolle Michael J. O’Donovan Motoneuronal Spinal Circuits in Degenerative Motoneuron Disease Frontiers in Molecular Neuroscience locomotion spinal muscular atrophy amyotrophic lateral sclerosis central pattern generator recurrent collaterals |
| title | Motoneuronal Spinal Circuits in Degenerative Motoneuron Disease |
| title_full | Motoneuronal Spinal Circuits in Degenerative Motoneuron Disease |
| title_fullStr | Motoneuronal Spinal Circuits in Degenerative Motoneuron Disease |
| title_full_unstemmed | Motoneuronal Spinal Circuits in Degenerative Motoneuron Disease |
| title_short | Motoneuronal Spinal Circuits in Degenerative Motoneuron Disease |
| title_sort | motoneuronal spinal circuits in degenerative motoneuron disease |
| topic | locomotion spinal muscular atrophy amyotrophic lateral sclerosis central pattern generator recurrent collaterals |
| url | https://www.frontiersin.org/article/10.3389/fnmol.2020.00074/full |
| work_keys_str_mv | AT melaniefalgairolle motoneuronalspinalcircuitsindegenerativemotoneurondisease AT michaeljodonovan motoneuronalspinalcircuitsindegenerativemotoneurondisease |