"Our findings may pave the way for development of drugs targeting the biological processes responsible for causing ALS, and leading to treatments or prevention of this currently fatal, incurable condition, " notes Pandey. "The fly model is an inexpensive and fast way to study ALS as well as many human diseases such as cancer, Alzheimer's disease and Parkinson's disease. Many basic biological processes are well conserved between humans and fruit flies, and nearly 75% of human disease-causing genes are believed to have a functional partner (homolog) in the fly that makes these small animals a highly tractable model system."
"These intriguing findings inspire us and other researchers to search for drugs that can make the defective FUS protein less toxic by targeting is RNA binding as a potential therapeutic intervention," noted Gavin Daigle (Graduate student in the Pandey lab and leading author of the manuscript).
According to the National Institutes of Health, Amyotrophic Lateral Sclerosis, sometimes called Lou Gehrig's disease, is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells (neurons) responsible for controlling voluntary muscles. The disease belongs to a group of disorders known as motor neuron diseases, which are characterized by the gradual degeneration and death of motor neurons. Motor neurons are nerve cells located in the brain, brainstem, and spinal cord that serve as controlling units and vital communication links between the nervous system and the voluntary muscles of the body. Messages from motor neurons in the brain (called upper motor neurons) are transmitted to motor neurons in the spinal cord (called lower motor neurons) and from them to particular muscles. In ALS, both the upper motor neurons and the lower motor neurons degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away (atrophy), and twitch (fasciculations). Eventual
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Louisiana State University Health Sciences Center