Prion

Prion diseases are caused by the accumulation of misfolded prion proteins in the brain. Two other prion diseases, Creutzfeldt-Jakob disease and Gerstmann-Straussler-Scheinker syndrome, may also occur as a result of variations of the PRNP gene, although some prion diseases occur in the absence of a genetic variation. Generally, prion disorders are characterized by long incubation periods and short clinical duration, which means the abnormal prions may accumulate for many years without causing symptoms (long incubation period), but once symptoms begin the disorder rapidly worsens.

Identified Prion Diseases are

• CJD (Creutzfeldt-Jakob Disease)
• NVCJD (New Variant Creutzfeldt-Jakob Disease)
• GSS (Gerstmann-Straussler-Scheinker Syndrome)
• FFI (Fatal Familial Insomnia)
• KRU (Kuru)