spongiform encephalopathies tagged posts

Scientists claim to have discovered the first new human prion in almost 50 years

Buildup in brain cells of the protein alpha-synuclein (dark spots) occurs in the neurodegenerative disorder Multiple System Atrophy (MSA).  Jensflorian/Wikimedia Commons, CC BY-SA 3.0

Buildup in brain cells of the protein alpha-synuclein (dark spots) occurs in the neurodegenerative disorder Multiple System Atrophy (MSA). Jensflorian/Wikimedia Commons, CC BY-SA 3.0

Prions are misfolded proteins that make copies of themselves by inducing others to misfold. By so doing, they multiply and cause disease. The resulting illness in this case is multiple system atrophy (MSA), a neurodegenerative disease similar to Parkinson’s. The study, published August 31 in Proceedings of the National Academy of Sciences, adds weight to the idea that many neurodegenerative diseases are caused by prions.

In the 1960s researchers led by Carleton Gajdusek at the National Institutes of Health transmitted kuru, a rare neurodegenerative disease found in Papua New Guinea, and Creutzfeldt–Jakob disea...

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