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Professor John Collinge
"There is no evidence that it would actually work once the disease is established"
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BBC's Sue Nelson reports
"It's hoped this research may one day offer a treatment that could slow the disease"
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The BBC's Richard Hollingham
"It ought to work in humans"
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Friday, 14 January, 2000, 01:08 GMT
CJD treatment 'draws closer'

CJD is thought to be transmitted to humans through infected beef
Treatment for the human form of BSE could be a step nearer with the discovery of a process which halts similar brain damage in mice, scientists have reported.

So-called spongiform diseases, such as BSE in cattle, or CJD in humans, are currently untreatable - causing irreversible, and inevitably fatal, brain damage.

But a report in The Lancet medical journal says experiments with mice have identified a process which can halt, and even reverse, the damage.

However, scientists at the Serono Pharmaceutical Research Institute have cautioned that they do not know if the process will achieve the same results in humans.

Sufferers' brain have spongy sections
Spongiform diseases are caused by the abnormal folding of nerve-tissue proteins, called prions, which lead to the build up of a destructive matter called "beta sheet".

The new process centres on a peptide - or protein component - that blocks the formation of the beta sheet.

Mice were infected with samples of CJD taken from people who had died of the disease, and then samples of the animals damaged brains were exposed to peptide in a laboratory.

They discovered that treatment with the peptide reversed the structure and properties of dangerous prions in the brains to a form similar to the original "safe" prion.

Dr Claudio Soto from the Serono Pharmaceutical Research Institute said: "There is still a lot of work to be done before we can use these peptides in the actual treatment of the disease.

"However, this concept may represent a novel approach."

In humans, CJD manifests itself with similar symptoms to more common illnesses like Alzheimer's Disease, such as fatigue, depression and memory problems.

The disease has been blamed on 'rogue' prions
The sufferer then develops shaking, and jerky movements, and eventually moves towards dementia and death.

The "beta-sheet breakers" used in the CJD experiments are also being tested as a potential treatment for Alzheimer's.

Last year, there were 17 confirmed CJD cases in the UK, a significant rise from the previous year, fuelling fears that the trend will continue to be sharply upwards as the true extent of CJD infection becomes apparent.

Sceptics suggested that the marked rise was due simply to better recording of cases.

However, research published in the British Medical Journal this week confirms that the rise is genuine, and free of statistical error.

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See also:

21 Dec 99 | Health
CJD: What is the risk?
15 Nov 99 | Health
Blood test for CJD created
18 Aug 99 | Health
Brain scans diagnose vCJD
23 Nov 99 | Health
13-year-old may have CJD
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