Contribution to antimitochondrial antibody production: Cleavage of pyruvate dehydrogenase complex‐E2 by apoptosis‐related proteases
Shuji Matsumura, Judy Van De Water, Hiroto Kita, Ross L. Coppel, Takao Tsuji, Kazuhide Yamamoto, Aftab A. Ansari, M. Eric Gershwin – 30 December 2003 – Patients with PBC produce a directed, specific response to a single immunodominant autoepitope of PDC‐E2 within the inner lipoyl domain. In contrast, immunized animals react to multiple epitopes and rarely recognize the inner lipoyl domain. In other autoimmune diseases, apoptosis plays a critical role in antigen presentation; the caspases and granzyme B are the key proteases in the generation of autoepitopes.