[Molecular mimicry between neurofilaments and titin as the basis for autoimmunity towards skeletal muscle in paraneoplastic myasthenia gravis].

Abstract

AIMS AND METHODS:

Autoantibodies against striated muscle proteins, particularly titin, characteristically distinguish thymoma-associated from non-paraneoplastic myasthenia gravis (MG) patients. However, the stimulus for this autoimmunity remains enigmatic since whole titin is not detectable in these tumors. Since Neurofilaments (NF) contain titin epitopes, MG-associated thymomas were investigated for NF expression using immunohistochemistry and Western blotting.

RESULTS:

In thymomas with cortical differentiation many of the neoplastic epithelial cells expressed medium molecular weight NF at the protein level. In sharp contrast, in medullary and mixed thymomas and in normal thymuses immunoreactivity with anti-NF antibodies was rare.

CONCLUSION:

The overexpression of NFs with titin epitopes by neoplastic cells in an inappropriate cortical environment may be the basis for the autosensitization of developing T-cells against titin in MG-associated cortical type thymomas.

• Authors: A Wilisch, A Schultz, A Greiner, T Kirchner, H K Müller-Hermelink, A Marx
• Journal: Verhandlungen der Deutschen Gesellschaft fur Pathologie (Verh Dtsch Ges Pathol) Vol. 80 Pg. 261-6 ( 1996) ISSN: 0070-4113 [Print] Germany
• Vernacular Title: Molekulares Mimikry zwischen Neurofilamenten und Titin als Grundlage der Autoimmunität gegen Skelettmuskulatur bei paraneoplastischer Myasthenia gravis.
• PMID: 9065023 (Publication Type: English Abstract, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Autoantibodies
• Connectin
• Membrane Proteins
• Muscle Proteins
• Neurofilament Proteins
• Receptors, Cholinergic
• TTN protein, human
• Protein Kinases

Topics

• Autoantibodies
• Connectin
• Humans
• Membrane Proteins (analysis, immunology)
• Muscle Proteins (analysis, immunology)
• Muscle, Skeletal (immunology, pathology)
• Myasthenia Gravis (immunology, pathology)
• Neurofilament Proteins (analysis, immunology)
• Paraneoplastic Syndromes (immunology, pathology)
• Protein Kinases (analysis, immunology)
• Receptors, Cholinergic (analysis, immunology)
• Thymoma (immunology, pathology)
• Thymus Neoplasms (immunology, pathology)