Inactivation of the IL-6 gene prevents development of multicentric Castleman's disease in C/EBPβ-deficient mice

Isabella Screpanti, Piero Musiani, Diana Bellavia, Manuela Cappelletti, Francesca B. Aiello, Marella Maroder, Luigi Frati, Andrea Modesti, Alberto Gulino, Valeria Poli

Research output: Contribution to journalArticle

Abstract

Castleman's disease is a lymphoproliferative disorder thought to be related to deregulated production of IL-6. We have previously shown that mice lacking the trans-acting factor C/EBPβ, a transcriptional regulator of IL-6 and a mediator of IL-6 intracellular signaling, develop a pathology nearly identical to multicenter Castleman's disease, together with increasingly high levels of circulating IL-6. We described here how the simultaneous inactivation of both (IL-6 and C/EBPβ genes prevents the development of pathological traits of Castleman's disease observed in C/EBPβ-deficient mice. Histological and phenotypic analysis of lymph nodes and spleen of double mutant mice did not show either the lymphoadenopathy and splenomegaly of the abnormal expansion of myeloid, B and plasma cell compartments observed in C/EBPβ-/- mice, while B cell development, although delayed, was normal. Our data demonstrate that IL-6 is essential for the development of multicentric Castleman's disease in C/EBPβ-/- mice.

Original languageEnglish
Pages (from-to)1561-1566
Number of pages6
JournalJournal of Experimental Medicine
Volume184
Issue number4
DOIs
Publication statusPublished - Oct 1 1996

ASJC Scopus subject areas

  • Immunology

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    Screpanti, I., Musiani, P., Bellavia, D., Cappelletti, M., Aiello, F. B., Maroder, M., Frati, L., Modesti, A., Gulino, A., & Poli, V. (1996). Inactivation of the IL-6 gene prevents development of multicentric Castleman's disease in C/EBPβ-deficient mice. Journal of Experimental Medicine, 184(4), 1561-1566. https://doi.org/10.1084/jem.184.4.1561