The Hindu : National : Work on to develop effective treatment for Prion diseases: Prusiner

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Sunday, Feb 18, 2007
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Work on to develop effective treatment for Prion diseases: Prusiner

Special Correspondent

`Discovery may lead to breakthroughs for Alzheimer's and Parkinson's'

— Photo: S. R. Raghunathan

HONOURED: Stanley Prusiner, Nobel laureate (centre), receives the scroll of honour from Venu Srinivasan, Chairman, T.S. Srinivasan Charitable Trust, in Chennai on Saturday. Professor K. Srinivas is with them.

CHENNAI: Will discovering a cure for Prion disease provide new approaches to treating Alzheimer's and Parkinson's Disease, asked Stanley Prusiner, the Nobel laureate who described Prions — misshapen proteins causing infections as seen in Mad Cow Disease.

Prof. Prusiner, whose work on the `proteinacious infective particle' is thought to have defined a new paradigm of disease, pointed out similarities between Prion Diseases and Alzheimer's and Parkinson's — all of them are caused by accumulation of protein in the brain and all of them have, currently, no treatment. He went on to speculate, therefore, if the treatment for Prion diseases were to be defined it would have some lessons for researchers working on treating the other two disorders as well.

Delivering the 27th T.S. Srinivasan Endowment Oration `On viruses, Genes and Mad Cows- Lessons from Prion Disease,' the Nobel laureate focused on Prion diseases in humans, including Kuru, Creutzfeldt-Jakob Disease (CJD), and those in animals, including Scrapie, Bovine Spongiform Encephalopathy (BSE) and Chronic Wasting Disease.

Prion disease

Revealing trends in on going research to treat Prion disease, Prof. Prusiner said work was on to formulate compounds that prevent Prion formation or stimulate dissolution at a later stage.

He made it clear while there were some signs of slow-down in progression of disease in experiments with mice, there was still a long way to go before effective treatment techniques could be developed.

Speaking to presspersons earlier, he said before the discovery of Prions, diseases were thought of as being caused by pathogens such as virus, fungi and bacteria. However, Prof. Prusiner's seminal work showed Prions, causing degenerative neural disorder, as different from all infection agents.

Only proteins

Prions were only proteins, with no nucleic acid. CJD, the human equivalent of mad cow disease, occurs when `rogue' proteins change shape and accumulate in the brain causing brain degeneration.

Prion diseases could occur as sporadic or spontaneous illness, as genetic or inherited conditions and also as communicable diseases, he said.

The Mad Cow Disease outbreak in Britain peaked in 1992, when about 50,000 cows were detected with BSE. The outbreak had occurred because of the changes introduced in the way offal from the cattle was treated before it was converted to meat and bone feed for cattle. There was clear evidence to prove transmission of BSE to humans, he said.

India has also started seeing cases of CJD, Prof. Krishnamoorthy Srinivas, founder-chairman, T.S. Srinivasan Centre for Clinical Neurosciences, VHS, said. The most common form was the spontaneous CJD illness that is seen in older people.

A study conducted by S.K. Shankar of NIMHANS recorded 78 cases in India over 32 years.

Broad ambit

Venu Srinivasan, industrialist and son of T.S. Srinivasan in whose memory the endowment lecture was instituted, said the dual areas of neurosciences and mental heath and the interface between them will form the broad ambit of the activities of T.S. Srinivasan Centre in the next five years.

N.S. Murali, honorary secretary, VHS, described the vision of VHS founder K.S. Sanjivi, in promoting community health.

The programme was sponsored by the T.S. Srinivasan Charitable Trust and the T.S. Srinivasan Centre for Clinical Neurosciences.

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