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New Way To Study Brain Disease Uses Neuro-Cells From Stem Cells.

EDINBURGH, U.K., November 20, 2017 -- Scientists here have developed a new system to study Creutzfeldt-Jakob disease in the lab, paving the way for research to find treatments for the fatal brain disorder.

The team have devised a method of studying the abnormal proteins (prions) responsible for the disease in specialized brain cells grown from stem cells.

The advance marks the first time scientists have infected human cells with the proteins in a lab.

Creutzfeldt-Jakob disease is a human disease like 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. These studies have yielded valuable information but the relevance of the findings for CJD has been unclear.

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, which are non-specialized cells that can transform into other cell types.

They infected these cells in a Petri dish with prions isolated from brain samples of CJD patients.

The infected astrocytes produced more prions and were able to infect neighboring healthy cells, the team found, something scientists had never been able to recreate in the lab before.

Experts say this marks a significant milestone in quest to better understand prion diseases in people, and could eventually aid the development of new therapies.

The research also demonstrates the potential of human stem-cell derived astrocytes to notably reduce, and in many instances, replace animal studies of human prion disease. The team said this could make a significant contribution to lowering the number of animals used in research in line with the principles of the 3Rs--i.e., reduction, replacement, refinement--which are a central tenet of the ethical use of animals in research.

Prion diseases cause brain damage that worsens rapidly over time and are invariably fatal. The most common form of prion disease in people is sporadic CJD, which occurs spontaneously in the population. A million people in the U.K. are affected each year.

A different form of the disease that is known as variant CJD can be acquired by consuming contaminated meat from animals with prion disease, such as BSE in cows. The disease has claimed 229 lives since it was first identified 20 years ago--of which 177 were from the UK--and it remains very rare. However, it has been estimated that as many as one in 2000 people in the UK could be carrying infectious prions without showing any symptoms.

Citation: Zuzana Krejciova et al., "Human stem cell-derived astrocytes replicate human prions in aPRNPgenotype--dependent manner," The Journal of Experimental Medicine, 2017; jem.20161547 DOI: 10.1084/jem.20161547


Contact: Siddharthan Chandran,

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Title Annotation:Advanced Stem Cell Technology
Publication:Stem Cell Research News
Geographic Code:4EUUK
Date:Dec 4, 2017
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