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Silencing The Cause Of Mad Cow Disease

Date:
December 4, 2006
Source:
Journal of Clinical Investigation
Summary:
BSE (more commonly known as mad cow disease) and CJD, a related disease in humans, are fatal neurodegenerative diseases caused by accumulation in the brain of an abnormally folded version (PrPsc) of a natural protein (PrPc). Although there are currently no therapies for the treatment of these diseases, a new study indicates that in mice silencing the gene encoding PrPc using a technique known as RNAi markedly delays the onset of PrPsc accumulation and disease.
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BSE (more commonly known as mad cow disease) and CJD, which is a related disease in humans that can occur spontaneously, be inherited, or be acquired (in some cases probably from cows with BSE), are fatal neurodegenerative diseases. It is thought that these diseases are caused by accumulation in the brain of an abnormally folded version (PrPsc) of a natural protein (PrPc). There are currently no therapies for the treatment of these diseases, making this an area of active investigation.

In a study appearing in the December issue of the Journal of Clinical Investigation, Alexander Pfeifer and colleagues from the University of Bonn, Germany, show that in mice silencing of the gene encoding PrPc suppresses the accumulation of PrPsc.

In vitro, silencing the gene encoding PrPc, using a technique known as RNA interference (RNAi), in already diseased neurons suppressed the accumulation of PrPsc. Similarly, in mice engineered to express the gene silencing therapeutic in a varying proportion of their neurons, the accumulation of PrPsc was markedly delayed, with the delay in accumulation of PrPsc being directly correlated with the proportion of neurons in the brain expressing the gene silencing therapeutic.

This study therefore provides hope that RNAi might provide a new approach for the development of a therapeutic to treat individuals and animals with neurodegenerative disorders such as CJD and BSE. However, as Qingzhong Kong from Case Western Reserve University says in an accompanying commentary "Much more research is needed before RNAi can be harnessed to treat..." these neurodegenerative disorders.


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The above post is reprinted from materials provided by Journal of Clinical Investigation. Note: Materials may be edited for content and length.


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Journal of Clinical Investigation. "Silencing The Cause Of Mad Cow Disease." ScienceDaily. ScienceDaily, 4 December 2006. <www.sciencedaily.com/releases/2006/12/061201180736.htm>.
Journal of Clinical Investigation. (2006, December 4). Silencing The Cause Of Mad Cow Disease. ScienceDaily. Retrieved August 30, 2015 from www.sciencedaily.com/releases/2006/12/061201180736.htm
Journal of Clinical Investigation. "Silencing The Cause Of Mad Cow Disease." ScienceDaily. www.sciencedaily.com/releases/2006/12/061201180736.htm (accessed August 30, 2015).

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