Multiple System Atrophy (MSA), a neurodegenerative disorder with similarities to Parkinson’s disease, is caused by a newly discovered type of prion, akin to the misfolded proteins involved in incurable progressive brain diseases such Creutzfeldt-Jakob Disease (CJD), according to two new research papers led by scientists at UC San Francisco.
The findings suggest new approaches to developing treatments for MSA, which currently has no cure, but also raise a potential concern for clinicians or scientists who come in contact with MSA tissue.
The new findings mark the first discovery of a human disease caused by a new prion in 50 years, since work at the National Institutes of Health in the 1960s showed that human brain tissue infected with CJD could transmit neurodegeneration to chimpanzees.
It wasn’t until 1982 that UCSF’s Stanley Prusiner, MD, isolated the causative agent for a related disease called scrapie, found in sheep, and characterized it as a prion, for “infectious protein.” He then determined that the same prion protein caused bovine spongiform encephalopathy (BSE), or “mad cow” disease, in cattle, and so-called “variant” CJD in humans who subsequently consumed BSE-contaminated beef or other tissues.
Read the entire UCSF news release HERE