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Prion Disease

Building on his revolutionary discovery in 1982, Dr. Stanley Prusiner and his colleagues at the IND have led the field in the study of prions, from their initial discovery to recent breakthroughs in their detection and treatment.

Prions (pronounced "PREE-ons") are formed from proteins normally present in the brain. As proteins turn into prions, they adopt an aberrant shape, which becomes self-propagating. And as prions accumulate, these abnormally folded, toxic proteins cause the brain to dysfunction. When prions spread throughout the brain, they cause irreversible damage. In each of the neurodegenerative diseases, a specific protein turns into a prion. The following lists some neurodegenerative diseases and the protein(s) that cause them.


Neurodegenerative Disease
     Causative Protein(s)   

Mad cow, scrapie, kuru, 
Creutzfeldt–Jakob disease 

        Prion protein

Alzheimer's disease 

        Abeta, tau

Parkinson's disease 


Frontotemporal dementia 


Amyotrophic lateral sclerosis
(Lou Gehrig's disease) 

        SOD1, TDP43, Fus

Huntington's disease