Human and Animal Spongiform Encephalopathies are Autoimmune Diseases: A Novel Theory and its Supporting Evidence

Abstract

Publisher Summary This chapter discusses the autoimmune diseases of human and animal spongiform encephalopathies. Spongiform encephalopathies (SEs) are fatal degenerative diseases of the central nervous system (CNS) that can occur both in animals and in humans. It is hypothesized in the chapter that a key step in the pathogenic process leading toward the development of various known forms of SE diseases involves the production of specific autoimmune antibodies against disease-causing prion protein (PrP sc ) and possibly other immunogenic macromolecules (proteins or non-proteins) present in the CNS. The autoimmune antibodies produced against PrP sc are not only directly responsible for the conversion of normal cellular prion protein (PrP c ) to PrP sc , but are also directly responsible for the accumulation of PrP sc in the CNS and other peripheral tissues. The production of autoimmune antibodies against PrP sc and/or other cellular macromolecules is also responsible for the antibody-mediated chronic autoimmune attack of PrP-expressing cells, such as neurons, contributing to the development of neurological lesions and clinical symptoms characteristic of various SE diseases.