Abstract
Cellular and molecular mechanisms of motor neuron death in amyotrophic lateral sclerosis: a perspective.
Highlights
Amyotrophic lateral sclerosis (ALS), which was described since 1869 by Jean Martin Charcot, is a devastating neurodegenerative disease characterized by the selective and progressive loss of upper and lower motor neurons of the cerebral cortex, brainstem and the spinal cord
The only known cause of the disease is associated with genetic mutations, mainly in the gene encoding superoxide dismutase 1 (SOD1) which is the most frequent case of familial ALS (FALS), representing about 20% of cases of FALS, albeit over one hundred mutations have been described in FALS
Both ALS types show similar histopathological and clinical characteristics, and in spite of numerous investigations using tissue from ALS patients and relevant advances in the design of several experimental models of motor neuron degeneration, it has not been possible to establish a clear cause-effect relationship regarding the loss of motor neurons or the motor alterations characteristic of the disease, such as fasciculations, spasticity, and progressive paralysis
Summary
Amyotrophic lateral sclerosis (ALS), which was described since 1869 by Jean Martin Charcot, is a devastating neurodegenerative disease characterized by the selective and progressive loss of upper and lower motor neurons of the cerebral cortex, brainstem and the spinal cord. Both ALS types show similar histopathological and clinical characteristics, and in spite of numerous investigations using tissue from ALS patients and relevant advances in the design of several experimental models of motor neuron degeneration, it has not been possible to establish a clear cause-effect relationship regarding the loss of motor neurons or the motor alterations characteristic of the disease, such as fasciculations, spasticity, and progressive paralysis.
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
