New type of prion may cause, transmit neurodegeneration

Multiple System Atrophy is Described as First New Human Prion Disease Identified in 50 Years

By Nicholas Weiler (UCSF News Center) on August 31, 2015

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.

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