Protein sheds insight into spread of vCJD to the brain
2 December 2011
A protein linked to the immune system could play a key role in helping scientists understand how vCJD spreads throughout the body.
The disease occurs when corrupted proteins - known as prions - accumulate in the spleen, lymph nodes and tonsils - before spreading to the brain where the disease destroys nerve cells.
When the follicular dendritic cells (red in image) expressed PrPC (blue), prions were able to replicate on the surface of these cells in the spleen and spread throughout the body. However, when PrPC expression was turned off only in these cells, the prions were not able to multiply and were destroyed by other cells. Neurones are shown in green.
Image: The Roslin Institute
Researchers found that they could thwart the spread of prions by preventing production of a protein in just one type of immune cell.
Stopping these cells from expressing this protein did not affect the regular function of the immune system.
The study by The Roslin Institute at the University of Edinburgh, which receives strategic funding from the Biotechnology and Biological Sciences Research Council (BBSRC), could lead to treatments to stop vCJD spreading to the brain and causing disease. However, any treatments would be viable only if scientists are able to find a way to diagnose the condition in its early stages.
Neil Mabbott, of The Roslin Institute at the University of Edinburgh, who led the study, said: "If we can find a way of stopping this protein from being expressed by specific immune cells then we could potentially block the spread of the disease to the brain. We also want to understand how cells are infected with vCJD in the first place, so that we can look at ways of stopping this from happening and find ways to diagnose the disease at its early stages."
The study, funded by BBSRC, looked at production of a protein - called PrPC - in specific immune cells.
These cells - follicular dendritic cells - act like spider's webs, attracting foreign particles, which can then be disposed of by the body's immune system.
The researchers found that when the cells expressed PrPC, corrupted proteins responsible for vCJD, known as prions, were able to replicate on the surface of these cells and spread throughout the body. However, when only these cells were prevented from producing PrPC, the prions were not able to multiply and were destroyed by other cells.
The study is published in the journal PLoS Pathogens.
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Tara Womersley, Press and PR Office, The University of Edinburgh
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