CJD study could lead to further research

SCIENTISTS HAVE developed a new system to study CreutzfeldtJakob disease in the laboratory, paving the way for research to find treatments for the fatal brain disorder.

The team led by the University of Edinburgh 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.

Dr James Alibhai, of the National CJD Research & Surveillance Unit at the University of Edinburgh, said the study could lead to new treatments, adding: “Creutzfeldt-Jakob disease was first reported almost 100 years ago but remains a universally fatal disease. We still have little understanding of what triggers the condition and how it is spread.”

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 generated brain cells called astrocytes from induced pluripotent stem cells – non-specialised cells that have the ability to transform into other cell types. They infected these cells 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.