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Gene Discovery Could Help ALS Patients
(Ivanhoe Newswire) -- Scientists are one step closer to understanding how to treat and repair diseases of the nervous system, such as amyotrophic lateral sclerosis (ALS), or Lou Gehrig’s disease.

According to a new study, researchers at the University of Rochester Medical Center and Harvard worked together to identify a gene in mice that plays a central role in the proper development of one of the nerve cells that degenerates in ALS.

The scientists focused on corticospinal neurons, nerve cells that connect the brain to the spinal cord. As the ends of these nerves go bad, patients lose the ability to control their muscles, the researchers said.

The team of scientists discovered a protein in mice known as Bhlhb5 is central to how stem cells in the brain ultimately become corticospinal motor neurons, one type of neuron that deteriorates in ALS. The researchers said understanding how the body determines the destiny of stem cells is crucial if doctors are going to use the cells to treat diseases like ALS, Parkinson’s and Huntington’s diseases and spinal cord injuries.

“We’re looking at how the most sophisticated portion of the brain, the neocortex, creates the right kind of neurons where and when they’re needed,” study author Jeffrey D. Macklis, M.D., D.HST of Harvard, was quoted as saying. “Understanding how our brain circuits are initially built is the first step to repairing or reversing many diseases of the nervous system.”

In lab tests, the study’s authors found when Bhlhb5 was knocked out in mice, cells that normally develop into neurons that connect the brain to the spinal cord did not do so. Those mice shared many traits with people afflicted with a neurological disease called hereditary spastic paraplegia. There is currently no cure or treatment for the disease, which affects about 10,000 to 20,000 Americans.

Scientists said they now plan to analyze the function of the human counterpart to the Bhlhb5 gene in patients.

SOURCE: Neuron, 2008; October 23, 2008