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Creutzfeldt Jakob disease dementia

Creutzfeldt Jakob disease dementia is a prion disease, which is 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 of them die within 1 ~ 2 years. Dementia is progressive. In the 1950s, sigurdsson (1954) studied sheep pruritus spongiform encephalopathy and proposed lentivirus infection. Prion protein gene (PRNP) is located in the short arm of chromosome 20. Prion protein exists in two forms in vivo. One is that the normal cell isomer (PrPC) is synthesized by endoplasmic reticulum and degraded rapidly with a short half-life of 3 ~ 6h. The structure of this protein is mainly composed of α Spiral composition, β Little folding. No pathogenic effect. Another pathogenic pruritus isomer (PrPSc), protein structure α There are few spirals, mainly composed of β Folding composition, slow translation processing, not easy to degrade, and cause disease by depositing in cell membrane, especially synaptic membrane.


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