Major ALS Breakthrough from Northwestern News on Vimeo.
Editor's Note: This story and headline were updated from their original form to indicate the Feinberg study detailing the root cause of ALS was published in 2011, and to highlight the influx of donations to the Les Turner ALS Foundation as a result of the Ice Bucket Challenge. North by Northwestern regrets not orginally making this clarification.
Feinberg School of Medicine published an article about the “ALS Ice Bucket Challenge” August 20, explaining the details of the challenge and what Northwestern has done to find a cure. They also credit the challenge with raising awareness and money for research. According to a Northwestern Medicine blog, donations to the Les Turner ALS Foundation grew more than 3,000 percent in the first two weeks of August compared to last year.
“Thanks to the ‘Ice Bucket Challenge,’ Northwestern’s Les Turner ALS Foundation donations have increased significantly compared to this time last year,” the article reads. “Money donated to this important cause makes it possible for Northwestern University and many other institutions to research ALS and work towards finding a cure for the disease.”
ALS is a fatal neurodegenerative disease that paralyzes its victims but does not typically affect the mind, leaving victims trapped in their own bodies.
Feinberg made a significant discovery in the realm of ALS treatment in 2011 with a study published about major proteins responsible for the development of the disease in patients.
The study's Senior Author Teppu Siddique, M.D., has been researching the disease and searching for its cause for more than 25 years. He said he was drawn to studying ALS because “it was one of the most difficult problems in neurology and the most devastating, a disease without any treatment or known cause.” The lead authors of the paper were Han-Xiang Deng, M.D., a Feinberg associate professor of neurology, and Wenjie Chen, senior research technologist in neurology.
Feinberg researchers discovered that a protein called ubiquilin2, which recycles damaged proteins in motor and cortical neurons and shuttles them off to be reprocessed, is the root of the problem.
People with ALS have malfunctioning ubiquilin2, which means that this protein and the other damaged proteins accumulate in motor neurons in both the spinal cord and the brain. These accumulations, which look like twisted yarn, cause neurons to degenerate.
All three forms of ALS involve this breakdown – hereditary (called familial); non-hereditary (called sporadic); and ALS that targets the brain, or ALS/dementia. About 90 percent of ALS cases are sporadic.
Siddique said the discovery “opens up a whole new field for finding an effective treatment for ALS.”