Key to possible Mad Cow Disease treatment
Friday November 20th, 2009
British scientists have made a discovery which could lead to an effective treatment for bovine spongiform encephalopathy (BSE), or Mad Cow Disease, it was announced last night.
BSE and its
human form Creutzfeldt Jakob disease (CJD) are caused by an abnormal infectious
form of a prion protein present on nervous system cells.
The human disease linked to BSE has affected a small number of people since it appeared in the late 1980s - but has had a devastating and fatal impact, often on young adults.
Professor Nigel Hooper of Leeds University, UK, and colleagues investigated why the prions become abnormal.
Professor Hooper explained: "We were looking at how the normal prion protein functions in cells and spotted that it was interacting with something. Some lateral thinking and deduction led us to Glypican-1 and when we carried out the experiment, we found we were right."
This protein, Glypican-1, plays an important role in the development of BSE. Its presence causes the number of abnormal prion proteins to increase. In experiments, the researchers found that when levels of Glypican-1 are reduced in infected cells, the levels of the abnormal prion also reduced.
This suggests that the protein functions as a scaffold bringing the two forms of the prion protein together, the team believes, and this contact causes normal prions to become infectious through mutation.
Findings are published today (November 20) in PLoS Pathogens.
"Now that we know the identity of one of the key molecules in the disease process, we may in the future be able to design drugs that target this," Professor Hooper said.
He added that it is possible that Glypican-1 is involved in other diseases of the nervous system. His team now plan to carry out further work towards developing treatments for both BSE and CJD.
"While initial experiments haven't shown any link with other neurodegenerative diseases like Alzheimer's, we're not yet completely ruling that out," Professor Hooper concluded.
Tags: Brain & Neurology | UK News
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