Published online Jan 7, 2016. doi: 10.3748/wjg.v22.i1.338
Peer-review started: May 8, 2015
First decision: July 14, 2015
Revised: August 15, 2015
Accepted: December 1, 2015
Article in press: December 1, 2015
Published online: January 7, 2016
Core tip: Environment influences immune functions. In this paper, we examine how environmental chemicals can trigger autoimmunity in an organ specific autoimmune disease, primary biliary cholangitis (PBC). PBC is liver specific autoimmune disease characterized by high titer of anti-mitochondrial autoantibodies directed against the E2 subunit of pyruvate dehydrogenase (PDC-E2) lipoyl domain. Here, we present experimental evidence from quantitative structure-activity relationship and animal models that xenobiotic modification of the PDC-E2 lipoyl domain could lead to loss of self-tolerance and is a pivotal event in the initial etiology of PBC in genetically susceptible hosts.