Title: antimitochondrial antibody
Long Title: IUPAC Gold Book - antimitochondrial antibody
DOI: 10.1351/goldbook.12895
Status: current

Definition
Autoantibody producing a mitochondrial staining pattern on sections of various tissues and on tumor cell monolayers.

Note
According to the fluorescence pattern, different subtypes can be differentiated. Antimitochondrial antibodies of the subtype 2 (AMA-M2) are directed against antigens of three related 2-oxo acid dehydrogenase complexes (e.g., the E2 subunit of the pyruvate dehydrogenase complex, PDC-E2) localized to the inner mitochondrial membrane. AMA-M2 is a specific marker of primary biliary cirrhosis (PBC).

Related Terms
- Autoantibody: https://goldbook.iupac.org//terms/view/12913
- primary biliary cirrhosis (PBC): https://goldbook.iupac.org//terms/view/13600

Source
- PAC, 2012, 84, 1113. 'IUPAC glossary of terms used in immunotoxicology (IUPAC Recommendations 2012)' on page 1126 (https://doi.org/10.1351/PAC-REC-11-06-03)

Other Outputs
- html: https://goldbook.iupac.org/terms/view/12895/html
- json: https://goldbook.iupac.org/terms/view/12895/json
- xml: https://goldbook.iupac.org/terms/view/12895/xml

Citation: Citation: 'antimitochondrial antibody' in IUPAC Compendium of Chemical Terminology, 5th ed. International Union of Pure and Applied Chemistry; 2025. Online version 5.0.0, 2025. 10.1351/goldbook.12895
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Accessed: 2026-07-14T02:54:42+00:00