Pocket Guide to Diagnostic Tests

Mitochondrial antibodies


Mitochondrial antibodies (AMA), serum

Negative (<1.0 U)



Physiologic Basis

Originally demonstrated using immunofluorescence approaches, antimitochondrial antibodies can now be detected using commercially available enzyme-linked immunosorbent assays (ELISAs). Although ELISAs are more practical, they are slightly less sensitive than immunofluorescence techniques. In AMA-negative patients with a high suspicion of primary biliary cirrhosis (PBC), antimitochondrial autoantibodies can be sought using recombinant autoantigens.


Increased in: Primary biliary cirrhosis (85–95%), chronic active hepatitis (25–28%), occasionally in CREST syndrome and other autoimmune diseases; lower titers in viral hepatitis, infectious mononucleosis, neoplasms, cryptogenic cirrhosis (25–30%).


Primarily used to distinguish PBC (antibodies present) from extrahepatic biliary obstruction (antibodies absent). The antigens recognized by AMA have been designated M1–M9. AMA from patients with PBC recognize the M2 antigen complex, which includes enzymes of the 2-pyruvate dehydrogenase (PDH-E2) and 2-oxoglutarate dehydrogenase.

The titer or levels of AMA do not indicate disease activity or prognosis in patients with PBC.

AMA subtype profiles do not predict prognosis in patients with PBC.

Hohenester S et al. Primary biliary cirrhosis. Semin Immunopathol 2009;31:283.  [PMID:19603170]

Mendes F et al. Antimitochondrial antibody-negative primary biliary cirrhosis. Gastroenterol Clin North Am 2008;37:479.  [PMID:18499032]

Muratori L et al. Antimitochondrial antibodies and other antibodies in primary biliary cirrhosis:

diagnostic and prognostic value. Clin Liver Dis 2008;12:261.  [PMID:18456179]

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