Reply: Antimitochondrial antibodies may be insufficiently specific to define primary biliary cirrhosis-like disease in mouse models

We appreciate the kind comments regarding our work on murine models of PBC but, with due respect, there are a number of points the authors should consider prior to conclusions. Firstly, our position has always been that AMA alone is insufficient to make a diagnosis of PBC in either humans or murine models; the diagnosis relies on evidence of autoimmune cholangitis identified on coded slides by a "blinded" pathologist (1). Importantly, cholangitis can be transferred to naive Rag recipients using CD8 T cells from dnTGFβRII mice demonstrating an autoimmune component, data consistent with flow cytometry of liver subpopulations and cytokine analysis. Third, AMA standardization uses not only ELISA, but also enzyme inhibition, immunoblotting, absorption, peptide microarrays, affinity purified antisera, and generation of large panels of mAbs (1), not just the ELISA data of Hohenester. In fact, definition and measurement of AMAs Hepatology