Subsequent research done at the NIH resulted in brain tissue being injected in chimpanzees, and transmission of Kuru-like disease to the chimpanzee. To gather information on the unusual characteristics of this disorder, Gadjusek traveled to New Guinea in the 1950’s and 1960’s to obtain specimens and do environmental evaluation. This group of people have, as a tribe, lost over 2,500 individuals to the disorder Kuru, mostly women and children. The Fore Tribe of New Guinea represents a unique population of people who have added much to the story. Review of this information may eventually show that this 30-fold increase in CJD was environmental as well as genetic. This group of people had an unusual dietary delicacy of sheep brains and sheep eyes. Subsequent studies in this population show that they have an amino acid variation in the 200th amino acid codon involving the described prion. In the 1930’s and 1940’s, an interesting subpopulation of Libyan Jews were studied and found to have a 30-fold increased risk for Creutzfeld Jacob Disease (CJD). This interesting disorder had no treatment and involved animals were often simply destroyed. Sheep affected with this disorder tend to scrape themselves repeatedly on poles or logs, thus rubbing the fur down to bare bone.
The history of prion disorders must start with scrapie, described over 200 years ago. Third, the protein catabolism associated with these diseases involves the newly described Ubiquitin system, knowledge of which will have tremendous impact on such broad-ranging topics as apoptosis (cell death), as well as future modalities for cancer therapy. Information on the protein chemistry and control and mechanisms involved with this family of diseases will offer a likelihood for therapeutic intervention. This disorder is really one of protein conformation and an unrelated, but somewhat similar, disorder in Alzheimer’s disease. Second, I believe that studying Mad Cow disease will offer an opportunity to study basic biochemical mechanisms that have far reaching implications. The world will soon see many cases of the latter disorder appearing in people. Subsequently the disorders have been described with some fanfare in cannibals from New Guinea in the form of Kuru and in the sporadic form of Creutzfeld Jacob Disease (CJD).
First, the prion disorders were initially thought to be exclusively veterinary, i.e. I have several reasons for studying these disorders. I would like to take an opportunity to review some of the prion-related disorders now popularized in the lay press, which include the so-called mad cow disease.
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