Wednesday, October 20, 2010

What is the Structure, Chemical Nature, Replication of Prions (The Puzzling Proteins)?



C. Gajdusek (1957) came across a mysterious disease in New Guinea tribals which was later named as Kuru and prepared neuropathological specimens from a person who died of Kuru. William Hadlow (1957) who was working on scrapie disease of sheeps and goats examined Gajduseks neuropathological specimens and observed remarkable similarities between the abnormalities found in brains of Kuru victim and the sheeps and goats dying of scrapie.

Similar observations were made by British investigators T. Alpher, D. Haig and M. Clark during 1966. In 1970s S.B,. Prusiner, a bichemist at the University of California (USA), with his coworkers initiated the isolation and identification of the infectious agent of scrapie. After exhaustive research for a decade, he in 1982 discovered that the disease is caused by a proteinaceous infectious particle which he christened as prions. S.B. Prusiner has been awarded Nobel Prize in 1997 for the discovery of prions.
Prions represent the other extreme from viroids. They are considered to be devoid of their own genetic material (DNA or RNA) and consist of just a single or two or three protein molecules i.e., a prion is merely an infectious protein. The discovery of prion, an infectious protein, has threatened the universally accepted concept that only the genetic material (DNA, in some cases RNA) is infectious.

The prions, at present, are considered to be the causative agents of some of the diseases of animals and humans such as Scrapie disease of sheeps and goats, Bovine spongiform encephalopathy in cattle (BSE or Mad cow diseases);. Kuru, Creutifeldt Jacob disease (CJD), Gerstmann-Strausslar syndrome (GSS), Low Gehrig disease, Parkinsons disease, Serite domentia and Multiple sclerosis in humans. In 1996, information available from England indicates that the prion causing Bovine spongiform encephalopathy (BSE) in cattle might infect humans, resulting in a variant of Creutzfeldt Jacob disease (CJD), called variant CJD or vCJD.

No comments:

Post a Comment

Related Posts with Thumbnails