Study of Rare, Terrible Brain Disease Yields Huge Find

Multiple system atrophy quickly destroys the brain
By Rob Quinn,  Newser Staff
Posted Sep 1, 2015 5:17 AM CDT
Updated Sep 1, 2015 6:33 AM CDT
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A brain-scanning MRI machine at Carnegie Mellon University in Pittsburgh.   (AP Photo/Keith Srakocic)

Multiple system atrophy, or MSA, is a rare and horrible disease that will destroy your brain and inevitably kill you, and the study of it has now yielded a major breakthrough in our understanding of brain diseases. Researchers have discovered that MSA is caused by a prion, a kind of deformed protein that spreads like a virus and is known to cause Creutzfeldt-Jakob disease in humans and bovine spongiform encephalopathy (aka mad cow disease) in cattle, reports the San Francisco Chronicle. The University of California, San Francisco scientists say alpha-synuclein is the first new kind of prion to be identified "since the discovery a half century ago that CJD was transmissible," they write in Proceedings of the National Academy of Sciences.

The scientists injected alpha-synuclein taken from the brains of 14 people who died from MSA into mice; NPR reports the animals were dead within about four months, and the dead creatures' brains "looked exactly like" those of the humans killed by MSA. The find lends support to the idea that "misfolded" prions could be behind more common brain diseases like Parkinson's and Alzheimer's. No cure currently exists for prion-caused diseases, which are generally spread by eating brains or eating animals that have eaten brains. But the find could help fight MSA, which acts like a more aggressive version of Parkinson's and affects up to 50,000 Americans at any time, NBC News reports. And while one prion expert says the research "suggests MSA does not transmit easily," study author Kurt Giles says "protein sticks very tightly to the stainless steel" and standard disinfecting may not kill prions, meaning it's possible MSA could be spread by contaminated surgical instruments. (Eating brains nearly wiped out this tribe in Papua New Guinea.)

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