Study Shifts Focus for Origin of Lou Gehrig’s Disease to Brain’s Motor Neurons

Lou Gehrig's disease, also known as amyotrophic lateral sclerosis (ALS), might damage muscle-controlling nerve cells in the brain earlier in the disease process than previously thought, according to research from the Cedars-Sinai Board of Governors Regenerative Medicine Institute in Los Angeles, California. The new findings, published in the November 19, 2014 issue of the Journal of Neuroscience, could shift researchers' attention from the spinal cord to the brain's motor cortex as the disease's initial point of dysfunction. "In this study, we show the exact progression of ALS in animals that have an inherited form of the disease, and we expose the brain's significant role in initiating the disease process, thought previously to originate in the muscle or spinal cord," said Clive Svendsen, Ph.D., professor and director of the Board of Governors Regenerative Medicine Institute. "We did this by selectively removing the disease-causing mutation just from the brains of ALS animals, and found that this alone had a big impact on disease initiation and progression." ALS causes weakness and gradual paralysis of muscles throughout the body, and although the timing and sequence of progression are unpredictable, the disease often begins in the arms or legs and eventually affects the breathing muscles in the chest. Patients generally live only three to five years after onset. The disease is known to affect motor neurons – nerve cells that control muscles – in the brain, brainstem, and spinal cord. It also inflicts damage in the nerve pathways extending from the spinal cord out to the muscles of the body. Breakdown of communication at the neuromuscular junctions – the points where nerve fibers connect to muscle fibers – is what ultimately leads to muscle weakness and failure.
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