Between 1980 and roughly 1996, about 750,000 cattle infected with BSE (bovine spongiform encephalopathy) were slaughtered for human consumption in Great Britain, and it is now clear that BSE, also known as “mad cow disease” is not merely a UK phenomenon, nor is it merely an economic nuisance. The sudden and explosive increase of BSE in recent Europe (1990-2000) may have been spread among cattle by the feeding of infected offal, but the majority of cases of naturally occurring prion diseases arise sporadically with no known cause. Thus, the most important problem to be solved is to elucidate the intrinsic chemical mechanism of the prion diseases which arise sporadically, i.e., we must answer the questions: What induces the conversion of normal prion protein into an abnormal isoform, and how the abnormal isofom forms without the infected offals ? Many years ago ALS (amyotrophic lateral sclerosis) patients were collectively found in the New Guinea and Papua islands, and its origin has been attributed to the subterranean water, which contains much Al3+ and Mn2+ ions. In Alzheimer’s disease specific region such as the hippocampus and the motor cortex contain elevated iron levels relative to normal, and abnormalities in brain iron metabolism have been described for several neurodegenerative disorders, including Alzheimer’s diseases, Parkinson’s disease, Huntington’s, and prion diseases. Investigations of scrapie, CJD, and chronic wasting disease clusters in Iceland, Slovakia and Colorado, respectively have indicated that the soil in these regions is low in copper and higher in manganese. Above facts suggest that the sporadic prion and other neurodegenerative diseases are closely related with the function of several transition-metal ions, and thus inorganic-biochemical perspectives are necessary in order to elucidate the chemical mechanisms of pathogenesis of these diseases. --Preface.
発行年
2006-10-20
出版者
Chemical Institute for Neruodegeneration, Yamagata University
Inorganic-Biochemical Perspectives of Sporadic Prion Diseases
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