Selective Vulnerability of Spinal and Cortical Motor Neuron Subpopulations in delta7 SMA Mice
| Autor: | Giorgio Battaglia, Marina Boido, Antonio Piras, Francesco Vagni, Denise Locatelli, Paolo d'Errico, Elena De Amicis, Valeria Valsecchi, Silvia Capra, Alessandro Vercelli |
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| Přispěvatelé: | D'Errico, Paolo, Boido, Marina, Piras, Antonio, Valsecchi, Valeria, De Amicis, Elena, Locatelli, Denise, Capra, Silvia, Vagni, Francesco, Vercelli, Alessandro, Battaglia, Giorgio |
| Jazyk: | angličtina |
| Rok vydání: | 2013 |
| Předmět: |
Cholinergic Neuron
lcsh:Medicine SMN1 Biology Motor Neuron Muscular Atrophy Spinal Mice Motor system medicine Animals Gliosis lcsh:Science Cerebral Cortex Mice Knockout Motor Neurons Biochemistry Genetics and Molecular Biology (all) Multidisciplinary Animal Pyramidal Cells lcsh:R Motor Cortex Gliosi Spinal muscular atrophy Anatomy Motor neuron SMA medicine.disease Spinal cord Survival of Motor Neuron 1 Protein Cholinergic Neurons Disease Models Animal medicine.anatomical_structure Agricultural and Biological Sciences (all) nervous system Spinal Cord Cerebral cortex Pyramidal Cell lcsh:Q Neuroscience Motor cortex Research Article |
| Zdroj: | PLoS ONE PLoS ONE, Vol 8, Iss 12, p e82654 (2013) |
| ISSN: | 1932-6203 |
| Popis: | Loss of the survival motor neuron gene (SMN1) is responsible for spinal muscular atrophy (SMA), the most common inherited cause of infant mortality. Even though the SMA phenotype is traditionally considered as related to spinal motor neuron loss, it remains debated whether the specific targeting of motor neurons could represent the best therapeutic option for the disease. We here investigated, using stereological quantification methods, the spinal cord and cerebral motor cortex of ∆7 SMA mice during development, to verify extent and selectivity of motor neuron loss. We found progressive post-natal loss of spinal motor neurons, already at pre-symptomatic stages, and a higher vulnerability of motor neurons innervating proximal and axial muscles. Larger motor neurons decreased in the course of disease, either for selective loss or specific developmental impairment. We also found a selective reduction of layer V pyramidal neurons associated with layer V gliosis in the cerebral motor cortex. Our data indicate that in the ∆7 SMA model SMN loss is critical for the spinal cord, particularly for specific motor neuron pools. Neuronal loss, however, is not selective for lower motor neurons. These data further suggest that SMA pathogenesis is likely more complex than previously anticipated. The better knowledge of SMA models might be instrumental in shaping better therapeutic options for affected patients. |
| Databáze: | OpenAIRE |
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