Scientists have developed a new system to study Creutzfeldt-Jakob disease in the laboratory, paving the way for research to find treatments for the fatal brain disorder.
The team have devised a method of studying the abnormal proteins responsible for the disease - called prions - in specialised brain cells grown from stem cells.
It is the first time scientists have been able to infect human cells with the proteins in the laboratory.
Creutzfeldt-Jakob disease (CJD) is a human disease similar to Bovine Spongiform Encephalopathy (BSE) in cows and Chronic Wasting Disease in deer.
Until now, the only way to study the human form of the disease has been in animals.
Efforts to investigate how prions are passed between brain cells have been hampered by an inability to replicate the proteins in human cells in the lab.
Researchers led by the University of Edinburgh generated brain cells called astrocytes from induced pluripotent stem cells - non-specialised cells that have the ability to transform into other cell types.
They successfully infected these cells in a dish with prions isolated from brain samples of CJD patients.
The infected astrocytes produced more prions and were able to infect neighbouring healthy cells, something scientists had never been able to recreate in the lab before.
Experts say this marks a significant milestone in the quest to better understand prion diseases in people, and could eventually aid the development of new therapies.
The study is published in the Journal of Experimental Medicine.
