The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy.
Spinal muscular atrophy (SMA), which is caused by inactivating mutations in the survival motor neuron 1 (SMN1) gene, is characterized by loss of lower motor neurons in the spinal cord. The gene encoding SMN is very highly conserved in evolution, allowing the disease to be modeled in a range of species. The similarities in anatomy and physiology to the human neuromuscular system, coupled with the ease of genetic manipulation, make the mouse the most suitable model for exploring the basic patho...Expand abstract
- Publisher copy:
- Copyright date:
If you are the owner of this record, you can report an update to it here: Report update to this record