Prion diseases are invariably fatal, but an experimental genetic treatment has dramatically extended the lifespans of infected mice
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An experimental therapy has dramatically extended the lives of mice infected with prions, which cause conditions like Creutzfeldt–Jakob disease . The news, announced by Sangamo Therapeutics, boosts hopes of developing treatments for these conditions. “Mice die very rapidly if they are not treated. The average lifespan of the mice that are treated is starting to extend over 500 days, which is kind of the normal lifespan,” saysthat makes other proteins of the same kind misfold too and join up to form damaging strand-like fibrils in cells. Fragments of fibrils spread the problem to other cells.This causes serious damage to the brain, with death usually occurring within a year of the first symptoms. “It’s a devasting disease,” says Fontenot.
Some forms of CJD are caused by eating contaminated food such as meat from cattle with BSE , also known as mad cow disease, or by surgical instruments or blood contaminated by prions. Others are due to mutations that make misfolding more likely. But with most cases of CJD, there is no clear cause – they may be a result of spontaneous misfolding.Get the most essential health and fitness news in your inbox every Saturday.that misfolds is one called PrP.
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