Identification and characterization of antimitochondrial autoantibodies in sera of patients with monoclonal gammopathies

Monoclonal gammopathies (MG) are defined by the accumulation of monoclonal immunoglobulins, a result of monoclonal B lymphocytes or plasma cell proliferative disorder. Only rarely do these antibodies cause an overt disease by binding to a specific autoantigen (e.g., factor VIII). In the present stud...

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Bibliographic Details
Published in:Immunology letters 1995-03, Vol.45 (3), p.163-166
Main Authors: Gilbrud, B., Weiss, P., Bakimer, R., Shoenfeld, Y.
Format: Article
Language:English
Subjects:
Antigen-Antibody Reactions
Antimitochondrial antibody
Autoantibodies - analysis
Autoantibodies - blood
Autoantibody
Catalysis
Epitopes - blood
Humans
Immunoglobulin kappa-Chains - blood
Immunoglobulin lambda-Chains - blood
Mitochondria - chemistry
Mitochondria - immunology
Monoclonal gammophathy
Paraproteinemias - blood
Paraproteinemias - immunology
Pyruvate dehydrogenase
Pyruvate Dehydrogenase Complex - antagonists & inhibitors
Pyruvate Dehydrogenase Complex - blood
Pyruvate Dehydrogenase Complex - immunology
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Summary:Monoclonal gammopathies (MG) are defined by the accumulation of monoclonal immunoglobulins, a result of monoclonal B lymphocytes or plasma cell proliferative disorder. Only rarely do these antibodies cause an overt disease by binding to a specific autoantigen (e.g., factor VIII). In the present study, sera from 100 patients with MG were screened for the presence of antibodies against the mitochondrial pyruvate dehydrogenase complex (PDH) — autoantibodies that are the hallmark of primary biliary cirrhosis (PBC). Anti-PDH antibodies were found in 6 patients, all asymptomatic. Using ELISA and immunoblotting methods, it was found that the titre of the anti-PDH antibodies was relatively low (average OD ± SD: 0.744 ± 0.529; PBC patients: 1.225 ± 0.291; P = 0.02). In each patient the autoantibodies were of both kappa and lambda chains, suggesting that they are of polyclonal origin and implying that in MG there is a significant production of polyclonal autoantibodies, in addition to monoclonal proliferation. Furthermore, in 5 of the 6 patients (83%) the anti-PDH antibodies did not recognize the E2 component of PDH (which is the major autoantigen in PBC) and did not inhibit the activity of PDH (which was inhibited by PBC autoantibodies). This is in concert with the fact that none of the patients developed liver disease and emphasizes the specificity of the anti-PDH autoantibodies associated with PBC.
ISSN:0165-2478
1879-0542
DOI:10.1016/0165-2478(94)00255-P