The disease resembles the symptoms of Parkinson’s disease.
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They have found that the disease is one of the few existent disorders that are linked to malfunctioning proteins, similar to the ones causing Creutzfeldt Jakob Disease, or more commonly known as the human form of “Mad Cow Disease”.
In a study featured in the journal Proceedings of National Academy of Sciences, UCSF researcher Dr. Stanley Prusiner and his colleagues discovered that multiple system atrophy is likely caused by misfolded brain proteins known as prions. The find lends support to the idea that “misfolded” prions could be behind more common brain diseases like Parkinson’s and Alzheimer’s.
A rare and incurable brain disease is caused by a misfolded brain protein similar to the one that causes mad cow disease, according to new research.
“Now we’ve conclusively shown that a new type of prion causes MSA”, said UCSF’s Kurt Giles, associate professor of neurology, IND researcher and senior author on the second of the two new studies.
Prusiner won the 1997 Nobel Prize in Medicine for his discovery of prions. The symptoms occur at about age 60, and about 90 percent of people who get the disease die within a year.
Because prion diseases can be transmitted through certain types of contact with infected tissue, the findings suggest a potential concern for doctors and researchers who work with tissue from MSA patients, the researchers said. “The TgM83+/- mice that were hemizygous for the mutant transgene did not develop spontaneous illness; in contrast, the TgM83+/+ mice that were homozygous developed neurological dysfunction”.
But Dr. Valerie Sim says that the study is not clear whether or not the disease is transmissible. Whether the same challenges hold for α-synuclein prions in MSA remains to be determined. Also, tools used for the deep brain stimulation treatment on MSA patients can be the source for contamination after a surgery if they are not properly decontaminated.
The authors write that clinicians and researchers should adopt much more stringent safety protocols when dealing with tissue from patients with MSA and other neurodegenerative diseases, many of which they believe may also be caused by prions. The disease can only be firmly diagnosed during post-death examination. The disease could potentially be transmitted to other patients if deep-brain stimulation equipment is reused. The harmful prions have a strong ability to cling hard to stainless steel wires, which might led to contamination as it’s impossible to clean or kill a protein, according to Giles. We’re advocating a precautionary approach.
Unlike the danger of bovine spongiform encephalopathy from contaminated beef, the researchers stress that there is no apparent risk of infection by MSA prions outside of specialized medical or research settings. This is significantly less time than the 120 days it takes for the disease to spread to a mouse model. Amanda Woerman, co-researcher from the University of California, San Francisco, said in a statement.
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These brain specimens were collected from the participants in a laboratory to be tested on mice and other healthy cells.
