This protein is also a minor antigen for antimitochondrial antibodies.

Gold standard diagnosis is antimitochondrial antibodies with liver biopsy as confirmation if showing florid bile duct lesions.

Presence of certain antibodies: antimitochondrial antibody (AMA), antinuclear antibody (ANA)

Morrison et al did describe a 52 year old woman with a weakly positive antimitochondrial antibody and Raynaud's disease, treated with immunosuppressive agents, who died secondary to PHT.

Primary biliary cirrhosis is suspected based on a medical history, physical exam, and lab tests that show high levels of the enzyme alkaline phosphatase and antimitochondrial antibodies (AMA) in the blood.