Go to JCI Insight
  • About
  • Editors
  • Consulting Editors
  • For authors
  • Publication ethics
  • Alerts
  • Advertising
  • Job board
  • Subscribe
  • Contact
  • Current issue
  • Past issues
  • By specialty
    • COVID-19
    • Cardiology
    • Gastroenterology
    • Immunology
    • Metabolism
    • Nephrology
    • Neuroscience
    • Oncology
    • Pulmonology
    • Vascular biology
    • All ...
  • Videos
    • Conversations with Giants in Medicine
    • Author's Takes
  • Reviews
    • View all reviews ...
    • Lung inflammatory injury and tissue repair (Jul 2023)
    • Immune Environment in Glioblastoma (Feb 2023)
    • Korsmeyer Award 25th Anniversary Collection (Jan 2023)
    • Aging (Jul 2022)
    • Next-Generation Sequencing in Medicine (Jun 2022)
    • New Therapeutic Targets in Cardiovascular Diseases (Mar 2022)
    • Immunometabolism (Jan 2022)
    • View all review series ...
  • Viewpoint
  • Collections
    • In-Press Preview
    • Commentaries
    • Research letters
    • Letters to the editor
    • Editorials
    • Viewpoint
    • Top read articles
  • Clinical Medicine
  • JCI This Month
    • Current issue
    • Past issues

  • Current issue
  • Past issues
  • Specialties
  • Reviews
  • Review series
  • Conversations with Giants in Medicine
  • Author's Takes
  • In-Press Preview
  • Commentaries
  • Research letters
  • Letters to the editor
  • Editorials
  • Viewpoint
  • Top read articles
  • About
  • Editors
  • Consulting Editors
  • For authors
  • Publication ethics
  • Alerts
  • Advertising
  • Job board
  • Subscribe
  • Contact
Top
  • View PDF
  • Download citation information
  • Send a comment
  • Share this article
  • Terms of use
  • Standard abbreviations
  • Need help? Email the journal
  • Top
  • Abstract
  • Version history
  • Article usage
  • Citations to this article

Advertisement

Research Article Free access | 10.1172/JCI115781

Human autoantibodies against desmoplakins in paraneoplastic pemphigus.

J R Oursler, R S Labib, L Ariss-Abdo, T Burke, E J O'Keefe, and G J Anhalt

Department of Dermatology, Johns Hopkins University, Baltimore, Maryland 21205.

Find articles by Oursler, J. in: JCI | PubMed | Google Scholar

Department of Dermatology, Johns Hopkins University, Baltimore, Maryland 21205.

Find articles by Labib, R. in: JCI | PubMed | Google Scholar

Department of Dermatology, Johns Hopkins University, Baltimore, Maryland 21205.

Find articles by Ariss-Abdo, L. in: JCI | PubMed | Google Scholar

Department of Dermatology, Johns Hopkins University, Baltimore, Maryland 21205.

Find articles by Burke, T. in: JCI | PubMed | Google Scholar

Department of Dermatology, Johns Hopkins University, Baltimore, Maryland 21205.

Find articles by O'Keefe, E. in: JCI | PubMed | Google Scholar

Department of Dermatology, Johns Hopkins University, Baltimore, Maryland 21205.

Find articles by Anhalt, G. in: JCI | PubMed | Google Scholar

Published June 1, 1992 - More info

Published in Volume 89, Issue 6 on June 1, 1992
J Clin Invest. 1992;89(6):1775–1782. https://doi.org/10.1172/JCI115781.
© 1992 The American Society for Clinical Investigation
Published June 1, 1992 - Version history
View PDF
Abstract

Recently, a previously unrecognized autoantibody mediated blistering disease, paraneoplastic pemphigus has been described. Paraneoplastic pemphigus is associated with lymphoid malignancies, thymomas, and poorly differentiated sarcomas. Serum of affected patients contain pathogenic autoantibodies that immunoprecipitate from normal keratinocytes a characteristic complex of four polypeptides with M(r) of 250, 230, 210, and 190 kD. As our preliminary studies indicated that the 250-kD and the 210-kD antigens comigrated with desmoplakins I and II, we investigated the possibility that autoantibodies against the desmoplakins were a component of this autoimmune syndrome. 11 sera from affected patients were tested by indirect immunofluorescence against desmosome containing tissues, immunoprecipitation of metabolically labeled keratinocytes, and Western immunoblotting of desmoplakins I and II that had been purified to homogeneity from pig tongue epithelium. By indirect immunofluorescence, 9 of 11 sera showed strong binding to epithelial and nonepithelial desmosomes, and 2 were weakly reactive. All 11 immunoprecipitated 250- and 210-kD bands of variable intensity that comigrated with bands identified by a murine monoclonal antidesmoplakin antibody, and immunoblotting confirmed binding of the serum autoantibodies to purified desmoplakins. This demonstrates that paraneoplastic pemphigus is the first human autoimmune syndrome in which autoantibodies against the desmoplakins are a prominent component of the humoral autoimmune response.

Images.

Browse pages

Click on an image below to see the page. View PDF of the complete article

icon of scanned page 1775
page 1775
icon of scanned page 1776
page 1776
icon of scanned page 1777
page 1777
icon of scanned page 1778
page 1778
icon of scanned page 1779
page 1779
icon of scanned page 1780
page 1780
icon of scanned page 1781
page 1781
icon of scanned page 1782
page 1782
Version history
  • Version 1 (June 1, 1992): No description

Article tools

  • View PDF
  • Download citation information
  • Send a comment
  • Share this article
  • Terms of use
  • Standard abbreviations
  • Need help? Email the journal

Metrics

  • Article usage
  • Citations to this article

Go to

  • Top
  • Abstract
  • Version history
Advertisement
Advertisement

Copyright © 2023 American Society for Clinical Investigation
ISSN: 0021-9738 (print), 1558-8238 (online)

Sign up for email alerts