Amyotrophic lateral sclerosis (ALS) is correlated with the cell’s excitability and function and has been posited as a plastic property that changes during cellular maturation, injury, and disease. A new research conducted by scientists from Wright State University shed light on how motor nerve cells (neurons) respond to motor neuron disease and could potentially provide new treatment options.
Motor neuron disease can lead to the death of motor nerve cells (neurons). It ultimately causes loss of muscle function due to paralysis. Currently, there is no cure for this and no effective treatment to halt or reverse the progression of the disease.
Most people with ALS die from respiratory failure, usually within 3 to 5 years from when the symptoms first appear. In a new study, scientists have discovered robust evidence that motor neurons change size throughout disease progression and that different types of neurons experience other changes.
The study suggests that motor neuron types that are more vulnerable to the disease – that is, they die first – increase in size very early in the disease before there are symptoms.
Scientists have also found that the neurons resistant to disease do not increase in size. According to scientists, such changes in the motor neurons can significantly impact their function and fate as the disease progresses.
Scientists identified and measured the size changes of motor neuron types in a mouse model of familial ALS. They then examined the motor neurons at every key stage of the disease to observe when and where these changes begin and how they progress throughout the disease.
Later on, scientists used specific antibodies as markers to bind to the structure of motor neurons so that they could be easily viewed under high-power microscopes, and a computer program performed the three-dimensional measurement of the size and shape of a motor neuron’s cell body.
Sherif M. Elbasiouny, the lead investigator on the research, said, “This research approach could be applicable not only to ALS but also to other neurodegenerative diseases, such as Alzheimer’s and Parkinson’s diseases. This new understanding could help us to identify new therapeutic targets for improving motor neuron survival.”
It is important to note that the research was carried out in only one mouse model, the most aggressive mouse model of ALS. Future work should focus on other mouse models of ALS to determine how relevant these results are likely to translate in human patients.
Journal Reference
- Dukkipati, S. S., Garrett, T. L., & Elbasiouny, S. M. (2018). The vulnerability of spinal motoneurons and soma size plasticity in a mouse model of amyotrophic lateral sclerosis. The Journal of Physiology, 596(9), 1723-1745. DOI: 10.1113/JP275498
