AI Article Synopsis
- Mice expressing human SOD1 serve as a model for ALS, demonstrating a pattern of motor dysfunction that progresses from the tail region to the head.
- Previous studies focused on the size of motor neurons (MNs) for assessing degeneration, but this research uses molecular markers to distinguish between alpha (α-) and gamma (γ-) MNs.
- By three months, α-MNs showed significant degeneration in both cervical and lumbar regions, while γ-MNs remained unaffected, indicating a specific pattern of vulnerability and confirming the idea of a spatial and temporal decline in MN health in SOD1 mice.
Article Abstract
Mice with transgenic expression of human SOD1 are a widely used model of ALS, with a caudal-rostral progression of motor impairment. Previous studies have quantified the progression of motoneuron (MN) degeneration based on size, even though alpha (α-) and gamma (γ-) MNs overlap in size. Therefore, using molecular markers and synaptic inputs, we quantified the survival of α-MNs and γ-MNs at the lumbar and cervical spinal segments of 3- and 4-month SOD1 mice, to investigate whether there is a caudal-rostral progression of MN death. By 3 months, in the cervical and lumbar spinal cord, there was α-MN degeneration with complete γ-MN sparing. At 3 months, the cervical spinal cord had more α-MNs per ventral horn than the lumbar spinal cord in SOD1 mice. A similar spatial trend of degeneration was observed in the corticospinal tract, which remained intact in the cervical spinal cord at 3- and 4- months of age. These findings agree with the corticofugal synaptopathy model that α-MNs and CST of the lumbar spinal cord are more susceptible to degeneration in SOD1 mice. Hence, there is a spatial and temporal caudal-rostral progression of α-MN and CST degeneration in SOD1 mice.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9137889 | PMC |
| http://dx.doi.org/10.3390/antiox11050983 | DOI Listing |
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