Creutzfeldt-Jakob disease dementia

Creutzfeldt-Jakob disease dementia is a prion (protein) disease and a degenerative disease of the central nervous system caused by prion protein transmission. The disease has a long incubation period and a short course of disease, and most often die within 1 to 2 years. Dementia is persistent and progressive. By the 1950s, Sigurdsson (1954) had studied pruritus spongiform encephalopathy in sheep and proposed lentiviral infection. The prion protein gene (PRNP) is located on the short arm of chromosome 20. Prion protein exists in two forms in the body. One is that the normal cell isomer (PrPc) is synthesized by the endoplasmic reticulum and has a short half-life of 3 to 6 hours. It rapidly degrades. No pathogenic effect. Another pathogenic pruritus isomer (PrPsc), which has very few protein structure helixes, is mainly composed of folds. Translation processing is slow and not easily degraded and causes disease in cell membranes, especially synaptic membranes.