Preface/Front Matter

Abstract

Four years ago, the first edition of Prion Biology and Diseases was published. Because a large number of recent studies on prions have greatly enhanced our understanding of these novel infectious pathogens, we decided to revise and modestly expand this book when the first edition became a “bestseller” and all the copies were sold.

In general, most scholars have come to accept the existence of prions. They believe that prions are different from all other infectious pathogens, and this view has aroused considerable interest. Bovine prions have spread from cattle with bovine spongiform encephalopathy (BSE) to humans in Great Britain and other parts of Europe and this continues to worry physicians, public health officials, and politicians. Despite the fact that molecular investigations of prions began only 21 years ago with the discovery of the protease-resistant fragment of the prion protein (PrP 27-30), there has been a steady increase in new knowledge, which is quite impressive. With the isolation of PrP 27-30, the application of modern molecular biological approaches, immunologic techniques, and structural biological studies became possible.

Like BSE in Europe, the continuing “spread” of chronic wasting disease (CWD) in free-ranging as well as captive deer and elk in the United States and Canada is of considerable concern. Whether CWD prions can be transmitted to humans either directly or first through sheep or cattle is unknown. The discovery of an 8-year-old cow with BSE in Alberta, Canada, led more than 30 countries to ban Canadian beef. Many questions have been raised