Do we understand CJD?

The effect of Creutzfeldt-Jakob disease (CJD) is so devastating that it eventually makes the brain look like a sponge: fine networks of fibres replace many of the cells. For this reason, CJD and related illnesses are also known as 'spongy brain diseases'. Many scientists believe that these diseases are caused by prions: agents made entirely out of protein (unlike bacteria and viruses, which also have genes). Infectious prions that cause CJD are variants of normal brain prion proteins. There is no cure for CJD.

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Structure of a prion.

What is 'new variant' CJD?

There is mounting evidence that a small number of people have contracted Creutzfeldt-Jakob disease after eating beef infected with bovine spongiform encephalopathy (BSE) or 'mad cow disease'. This 'new variant' CJD seems to affect younger people. Recent studies indicate that it causes changes in the brain that are more similar to BSE than other forms of CJD.

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CJD in humans has been linked to BSE (mad cow disease) in cattle.

BSE to CJD?

Creutzfeldt-Jakob disease (CJD) in humans, BSE in cows and scrapie in sheep are related diseases. The BSE epidemic probably resulted from cattle eating feed containing meat prepared from infected animals. Since 1989, adult cattle tissues (such as brain and spinal cord) that may be infected with BSE have been banned from human food in the UK. But because of the long incubation period of these diseases (possibly as much as 10 to 20 years), some people might have been infected before the ban was introduced.

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Structure of a prion.

 

Principal Funder:

Wellcome trust

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