Differential expression of perforin in muscle-infiltrating T cells in polymyositis and dermatomyositis.

N Goebels; D Michaelis; M Engelhardt; S Huber; A Bender; D Pongratz; M A Johnson; H Wekerle; J Tschopp; D Jenne; R Hohlfeld

doi:10.1172/JCI118749

Department of Neuroimmunology, Max-Planck-Institute of Psychiatry, Martinsried, Germany.

Find articles by Goebels, N. in: JCI | PubMed | Google Scholar

Department of Neuroimmunology, Max-Planck-Institute of Psychiatry, Martinsried, Germany.

Find articles by Michaelis, D. in: JCI | PubMed | Google Scholar

Department of Neuroimmunology, Max-Planck-Institute of Psychiatry, Martinsried, Germany.

Find articles by Engelhardt, M. in: JCI | PubMed | Google Scholar

Department of Neuroimmunology, Max-Planck-Institute of Psychiatry, Martinsried, Germany.

Find articles by Huber, S. in: JCI | PubMed | Google Scholar

Department of Neuroimmunology, Max-Planck-Institute of Psychiatry, Martinsried, Germany.

Find articles by Bender, A. in: JCI | PubMed | Google Scholar

Department of Neuroimmunology, Max-Planck-Institute of Psychiatry, Martinsried, Germany.

Find articles by Pongratz, D. in: JCI | PubMed | Google Scholar

Department of Neuroimmunology, Max-Planck-Institute of Psychiatry, Martinsried, Germany.

Find articles by Johnson, M. in: JCI | PubMed | Google Scholar

Department of Neuroimmunology, Max-Planck-Institute of Psychiatry, Martinsried, Germany.

Find articles by Wekerle, H. in: JCI | PubMed | Google Scholar

Department of Neuroimmunology, Max-Planck-Institute of Psychiatry, Martinsried, Germany.

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

Department of Neuroimmunology, Max-Planck-Institute of Psychiatry, Martinsried, Germany.

Find articles by Jenne, D. in: JCI | PubMed | Google Scholar

Department of Neuroimmunology, Max-Planck-Institute of Psychiatry, Martinsried, Germany.

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

Published in Volume 97, Issue 12 on June 15, 1996
J Clin Invest. 1996;97(12):2905–2910. https://doi.org/10.1172/JCI118749.
© 1996 The American Society for Clinical Investigation

Published June 15, 1996 - Version history

Polymyositis (PM) and dermatomyositis (DM) are the prototypical inflammatory diseases of skeletal muscle. In PM, CD8+ T cells invade and destroy muscle fibers, whereas humoral effector mechanisms prevail in DM. We studied the expression of the cytotoxic mediator perforin in inflammatory cells in PM and DM muscle by semiquantitative PCR, immunohistochemistry and confocal laser microscopy. Similar levels of perforin mRNA were expressed in PM and DM, and abundant perforin-expressing CD3+CD8+ and CD3+ CD4+ T cells were observed in both diseases. However, there was a striking difference in the intracellular localization of perforin. In DM, perforin was distributed randomly in the cytoplasm of the inflammatory T cells. In contrast, 43% of the CD8+ T cells that contacted a muscle fiber in PM showed perforin located vectorially towards the target muscle fiber. The results suggest (a) that the random distribution of perforin in the cytoplasm of muscle-infiltrating T cells observed in DM reflects nonspecific activation, and (b) that the vectorial orientation observed only in PM reflects the specific recognition via the T cell receptor of an antigen on the muscle fiber surface, pointing to a perforin- and secretion-dependent mechanism of muscle fiber injury.

Version 1 (June 15, 1996): No description

Differential expression of perforin in muscle-infiltrating T cells in polymyositis and dermatomyositis.

N Goebels, D Michaelis, M Engelhardt, S Huber, A Bender, D Pongratz, M A Johnson, H Wekerle, J Tschopp, D Jenne, and R Hohlfeld

Article tools

Metrics

Go to

Differential expression of perforin in muscle-infiltrating T cells in polymyositis and dermatomyositis.

N Goebels, D Michaelis, M Engelhardt, S Huber, A Bender, D Pongratz, M A Johnson, H Wekerle, J Tschopp, D Jenne, and R Hohlfeld

Article tools

Metrics

Go to

Sign up for email alerts