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Hope for ALS therapy 

Researchers find a key to how ALS kills cells.

click to enlarge Steve Gleason has worked hard to raise awareness about ALS and raise money for research. - CHERYL GERBER
  • Cheryl Gerber
  • Steve Gleason has worked hard to raise awareness about ALS and raise money for research.

Researchers have found a naturally occurring protein that could open a pathway for treating amyotrophic lateral sclerosis (ALS).

A research paper written by J. Gavin Daigle, a doctoral candidate at the Louisiana State University Health New Orleans School of Graduate Studies, was published this month in the online journal Acta Neurpathologica.

Pur-alpha, a protein that is present in neuron cells, could hold the key to slowing neurodegeneration in patients with ALS, the paper concluded.
ALS is a neurodegenerative disease that affects nerve cells in the brain and spinal cord that signal and control muscles. When those neuron cells die, the brain no longer can communicate or control muscles. Patients lose their ability to move, speak and sometimes swallow or breathe.

There is no cure.

Daigle and researchers from LSU and universities in Pittsburgh and Philadelphia, Pennsylvania, Norfolk, Virginia, and Bethesda, Maryland, used neuronal cell lines from ALS Patients, which all have gene mutations, and examined the mechanisms that cause neurodegeneration. They found the degeneration was triggered when cells produced “stress granules” to protect them against damage from heat, disease and other stresses — a normal process — but were unable to break down and expel the granules as healthy cells do once the trauma ended, causing the cells to die. Increasing Pur-alpha in such cells helps with both actions, the paper said.


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