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Amyotrophic Lateral Sclerosis (ALS) is motor neuron disease associated with the death of motor nerve cells ( neurons ). Progressive loss of muscle function, paralysis and death due to inability to breathe are the common symptoms. Currently, there is no cure for ALS and no effective treatment to stop or reverse the progression of the disease.
Studies in animal models of ALS showed inconsistencies in the changes in the size of motor neurons. Motor neurons change size over the course of disease progression and different types of neurons experience different changes. Those that are more vulnerable to the disease increase in size very early in the disease before any symptoms. Other motor neuron types that are more resistant to the disease die last and do not increase in size. These changes in the size of the motor neurons have effect on their function and their fate as the diseases progresses.
Motor neurons might alter their characteristics as a response to the disease to compensate for loss of function. However these changes can lead to the neuron’s early death. The most vulnerable motor neurons undergo unique changes that might impact their ability to survive.
The research conducted by Wright State University involved identifying and measuring size changes of motor neuron types in a mouse model of familial ALS. The motor neurons were examined at every key stage of the disease to observe when and where these changes begin, and how they progress. The research approach could be applicable to other neurodegenerative diseases, such as Alzheimer’s and Parkinson’s diseases.
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