The immune system is involved. There is partial MHC restriction in both human and animal disease. The immune reaction seen in these spongiform diseases can be explained by superantigen activity, Bastian says. This results in some immunoglobulin production, but only transiently. The major effect is clonal deletion of T cells, resulting in a state of immune tolerance.
Autoantibodies can also form.
Bastian proposes that, in spongiform diseases, PrP presumably acts as a superantigen. The question, he says, is what is producing the superantigen. He proposes that it is a bacterium. He says he is especially excited by the fact that the mollicutes cause superantigen production, adding that it is noteworthy that inclusion body myositis, a condition in which prions are seen , is an established superantigen disease. The pathology in the monkeys is identical to that seen in the recent human cases of CJD in Great Britain and appears to confirm that these cases were the result of transmission to humans of BSE, Bastian says.