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MITOCHONDRIAL ANTIBODIES IN PRIMARY BILIARY CIRRHOSIS
Open accessAbstract
The antigen reacting with complement-fixing antibodies in the sera of patients with primary biliary cirrhosis was localized predominantly in the mitochondrial fraction of tissue homogenates obtained by differential centrifugation. Purified mitochondrial preparations had a high content of the antigen whereas purified lysosomes failed to fix complement with PBC sera. Analysis of a number of fractionation experiments showed a high correlation between antigen content and the mitochondrial enzyme succinic dehydrogenase in all fractions. There was much poorer correlation with lysosomal and micrososomal enzyme markers. The patterns of staining obtained with a fluorescein conjugate of IgG from a PBC patient closely paralleled those obtained with a histochemical method for the demonstration of succinic dehydrogenase, further confirming the mitochondrial localization of the antigen. Staining was brightest in cells containing mitochondria with well-developed cristae. Studies on mitochondria fragmented by osmotic lysis, hexane, lysolecithin, and ultrasound suggest that the antigen is associated with the mitochondrial inner membranes.
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“PBC is characterised serologically by autoantibodies specific for mitochondrial, nuclear and centromere antigens, some of which are unique to PBC.66–78”
From The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines (2018) · cites this paper as [69]
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“Unlike in many other autoimmune diseases, these autoantibodies are, as a result of their sensitivity and specificity, extremely useful in diagnosis and have contributed significantly to the decline in the need for liver biopsy, at least for the purposes of diagnosis.”
From The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines (2018) · cites this paper as [69]
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“They are present in ~95% (mitochondrial) and ~30% (nuclear) of patients.”
From The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines (2018) · cites this paper as [69]
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References 30 · Cited by 201
Metadata
- Authors
- Berg, P. A., Doniach, D., & Roitt, I. M.
- Affiliations
- From the Rheumatology Research Department, Middlesex Hospital Medical School, London, England
- Venue
- The Journal of Experimental Medicine
- Publisher
- Rockefeller University Press
- Published
- 1967-08-01
- Volume
- 126
- Issue
- 2
- Pages
- 277-290
- ISSN
- 0022-1007 (ISSN-L)
- 1540-9538
- PMC
- PMC2138313
- OpenAlex
- W2159295244
- Cited by
- 201
- References
- 30
- Citations by year
- 2025: 2 · 2023: 2 · 2020: 2 · 2018: 2 · 2014: 2 · 2013: 4 · 2012: 3
- FWCI
- 15.75
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- The Journal of Experimental Medicine (publishedVersion)
- PubMed Central (publishedVersion, cc-by-nc-sa)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Pharmacology, Toxicology and Pharmaceutics › Pharmacology › Drug-Induced Hepatotoxicity and Protection
- Keywords
- primary biliary cirrhosis, antimitochondrial antibodies, complement-fixing antibodies, mitochondrial antigens, succinate dehydrogenase, inner mitochondrial membrane, mitochondrial cristae, lysosomes, microsomes
- MeSH terms
- Antibodies, Antigen-Antibody Reactions, Antigens, Cell Membrane, Centrifugation, Complement Fixation Tests, Clinical Enzyme Tests, Fluorescent Antibody Technique, Humans, Immunoassay, Liver Cirrhosis, Biliary, Mitochondria, Staining and Labeling, In Vitro Techniques
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- CoChat. (2026). Citation check: MITOCHONDRIAL ANTIBODIES IN PRIMARY BILIARY CIRRHOSIS. https://app.cochat.ai/papers/10.1084/jem.126.2.277
- The paper
- Berg, P. A., Doniach, D., & Roitt, I. M. (1967). MITOCHONDRIAL ANTIBODIES IN PRIMARY BILIARY CIRRHOSIS. The Journal of Experimental Medicine. https://doi.org/10.1084/jem.126.2.277
