Cell-Based Model Advances Research for Prion Disease in Humans

The Physicians Committee

Cell-Based Model Advances Research for Prion Disease in Humans

December 1, 2017

Image Source: NC3Rs

Study in a Sentence: By using stem cells to make astrocytes (a key support cell in the brain), scientists were able to develop a new cell culturing system using astrocytes to support the production of prions, the causative agent of the fatal neurodegenerative disorder Creutzfeldt-Jakob disease (similar to mad cow disease).

Healthy for Humans: This new research technique mimics the production of prions in patients. Thus, it allows the cell-to-cell transmission of this disease to be studied outside of the body. It can also serve as a drug screening tool to identify new potential therapeutic agents for the disease.

Redefining Research: This cell-based model allows the production of prions to be studied in a human-relevant model, bypassing the need to study its transmission in animal models. Thus, it is a significant contribution towards reducing and replacing the use of animals for studying human prion disease.

Krejciova Z, Alibhai J, Zhao C, et al. Human stem cell-derived astrocytes replicate human prions in a PRNP genotype-dependent manner. J Exp Med. Published online November 15, 2017. doi: 10.1084/jem.20161547.

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