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Multiple System Atrophy Caused By Mad Cow Disease-Like Proteins, UCSF Study Claims

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The rare and incurable brain disorder known as multiple system atrophy (MSA) affects anywhere between 15,000 and 50,000 Americans at any given moment and now researchers think they know the cause of the fatal disease.

According to the findings of a recently published study led by Dr. Stanley Prusiner, director of the University of California San Francisco’s Institute for Neurodegenerative Diseases, MSA is likely caused by abnormally folded proteins known as prions, which cause other proteins to fold in a similarly abnormal fashion — the results of which can be devastating.

The researchers discovered a new prion, known as alpha-aynuclein, which they believe to be responsible for causing MSA. According to the researchers, their newly discovered prion marks the first to be found in 50 years. Alpha-aynuclein are similar to the proteins which cause the human form of mad cow disease, Creutzfeld-Jakob Disease (CJD).

In the study, which was published in the journal Proceedings of National Academy of Sciences (PNAS), Dr. Prusiner and his colleagues examined the brains of 14 individuals previously diagnosed with the rare disease.

Using specimens derived from the diseased brains they were studying, the researchers found that they were able to transmit the disease to laboratory mice and other healthy cells by directly injecting them with the infectious specimens. But while this notion might stir concern in some, ABC News reports that the associate director of Colorado State University’s Prion Research Center, Mark Zabel, has written off the disease as transmissible but incapable of inducing an epidemic.

When the infectious specimens were used to infect healthy cells contained within lab dishes, they found that the infection altered the function of the healthy cells and caused symptoms reminiscent of Parkinson’s disease in mice. MSA is initially difficult to distinguish from Parkinson’s as the symptoms are similar and MSA can only be definitively diagnosed through postmortem examination of the brain.

Brains infected with MSA exhibit an inappropriate buildup of alpha synuclein and as the mice infected with the disease were found to exhibit an inappropriate accumulation of the same protein within their brains following infection, the researchers believe that they’ve found their confirmation.

Kurt Giles, a UCSF researcher and expert in neurology who worked on the study, was quoted by NBC News as having said that buildup of alpha-synuclein in the brains of mice has “conclusively shown” that a new type of prion is the cause of MSA.

Now we’ve conclusively shown that a new type of prion causes MSA

A prion expert at the University of Alberta in Canada who was not involved in the study, Dr. Valerie Sim, was quoted by ABC News as having noted in a statement that the UCSF study “does not demonstrate human-to-human transmission” and that it actually suggests that the disease “does not transmit easily.”

It is important to state that this study does not demonstrate human-to-human transmission. In fact, it suggests MSA does not transmit easily (…) Some of the message taken from this study is fear. It’s important to avoid fear

With all this in mind, the study’s authors are cautioning doctors to be particularly careful when performing “deep brain stimulation” due to the possibility, however minuscule, of infecting others with MSA.

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