The Underlying Genetic Cause of Prion Diseases

The human genome contains millions of base pairs that are successfully transcribed and translated to produce the necessary gene products for life. Sometimes translated protein products are susceptible to mutations that render them deadly for human disease. In the past decade, prion diseases such as cattle mad cow disease, human Kur's disease, sheep scrapie, etc. have become more prominent in private life (Araújo, 2013). Prion protein is a protein that may have catastrophic consequences by mutation. The underlying genetic cause of prion diseases is being studied to understand how polymorphisms of prion protein genes (PRNPs), host factors, and mutations cause severe physiological damage

Prion disease or infectious spongiform encephalopathy (TSE) is a unique, lethal neurodegenerative disease with infectious, inherited or sporadic causes. Prion diseases affect humans (eg Creutzfeldt-Jakob disease, Gerstmann-Straussler-Scheinker syndrome and lethality familial insomnia), nonhuman (eg bovine spongiform encephalopathy, chronic consuming cervical disease, and sheep And goats) Scrapie: Regardless of the species affected, prion disease causes a progressive decrease in neurocognition after a long incubation period Without effective prion therapy, most human patients will receive 14 months from diagnosis I will die within.

Prion disease is a group of neurodegenerative diseases caused by prions, "protein infectious particles". For some background, the first thing to see is the introduction of prions. Prion diseases are caused by prion proteins in misfolded form, also known as PrP. In addition to humans, these diseases affect many different mammals - for example, sheep have scrapie, cows have mad cow disease, and deer has chronic wasting disease. Human-type prion diseases are usually caused by Creutzfeldt-Jakob disease (CJD), fatal familial insomnia (FFI), Geltmann-Straussler-Scheinker syndrome (GSS), rickets disease and variable protease susceptible prion diseases (VPSPr) Since all of these diseases are caused by slightly different versions of the same protein, we call them all prion diseases.