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Neuron Killers

Misfolded, clumping proteins evade conviction, but they remain prime suspects in neurodegenerative diseases

As open-and-shut cases go, Alzheimer’s disease should top the list. The victim is clear. Suspects are in custody. Wherever neurons die due to Alzheimer’s disease, a protein known as amyloid-beta is always found at the scene of the crime, hanging around in large, tough gangs called plaques. Parkinson’s and Huntington’s diseases; amyotrophic lateral sclerosis (which goes by its initials ALS or the alias Lou Gehrig’s disease); and prion diseases, such as scrapie in sheep, mad cow disease in cattle and Creutzfeldt-Jakob disease in humans, all have similar stories.

Scientific investigators have pieced together this much: A seemingly mild-mannered brain protein falls in with a bad crowd, the corrupted protein and its cronies gang up and mob violence results in the death of a brain cell. It’s a scene repeated over and over again in different neighborhoods of the brain, by different proteins, but all with the same result — the death of neurons and rise of disease.

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