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Updated: Jul 22, 2026

The CYP2D6 Animal Model: How to Induce Autoimmune Hepatitis in Mice
Published on: February 3, 2012
Two cytochromes P450 are major hepatocellular autoantigens in autoimmune polyglandular syndrome type 1
M G Clemente1, A Meloni, P Obermayer-Straub
1Istituto di Clinica e Biologia dell'età evolutiva, Università degli Studi di Cagliari, Italy.
Background & Aims:
Liver disease has been described in 10%-15% of patients with autoimmune polyglandular syndrome type 1 (APS-1). After the discovery of cytochrome P450 1A2 (CYP1A2) as a hepatocellular autoantigen in liver-kidney microsomal autoantibody (LKM)-positive patients with APS-1, the investigation of antiliver antibodies was extended to 11 Sardinian patients with APS-1.
Methods:
Indirect immunofluorescence and Western blotting analysis were performed to study the antiliver antibodies.
Results:
Immunofluorescence revealed LKM antibodies in 3 patients with APS-1, 1 of whom died of fulminant hepatitis. Western blotting showed a liver microsomal protein band of approximately 51 kilodaltons in the LKM-positive sera of these 3 patients. Western blotting performed with recombinant cytochrome P450 enzymes allowed the identification of CYP2A6 as a specific target antigen.
Conclusions:
LKM antibodies in APS-1 sera are specifically directed against CYP1A2 or CYP2A6, but their diagnostic and prognostic significance for liver disease remain to be determined.
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