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

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Interleukin-6
Giant Lymph Node Hyperplasia
Genes
B-Lymphocytes
Trans-Activators
Lymphoproliferative Disorders
Splenomegaly
Myeloid Cells
Plasma Cells
Multi-centric Castleman's Disease
Spleen
Lymph Nodes
Pathology

ASJC Scopus subject areas

  • Immunology

Cite this

Screpanti, I., Musiani, P., Bellavia, D., Cappelletti, M., Aiello, F. B., Maroder, M., ... 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

Inactivation of the IL-6 gene prevents development of multicentric Castleman's disease in C/EBPβ-deficient mice. / Screpanti, Isabella; Musiani, Piero; Bellavia, Diana; Cappelletti, Manuela; Aiello, Francesca B.; Maroder, Marella; Frati, Luigi; Modesti, Andrea; Gulino, Alberto; Poli, Valeria.

In: Journal of Experimental Medicine, Vol. 184, No. 4, 01.10.1996, p. 1561-1566.

Research output: Contribution to journalArticle

Screpanti, I, Musiani, P, Bellavia, D, Cappelletti, M, Aiello, FB, 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, vol. 184, no. 4, pp. 1561-1566. https://doi.org/10.1084/jem.184.4.1561
Screpanti, Isabella ; Musiani, Piero ; Bellavia, Diana ; Cappelletti, Manuela ; Aiello, Francesca B. ; Maroder, Marella ; Frati, Luigi ; Modesti, Andrea ; Gulino, Alberto ; Poli, Valeria. / Inactivation of the IL-6 gene prevents development of multicentric Castleman's disease in C/EBPβ-deficient mice. In: Journal of Experimental Medicine. 1996 ; Vol. 184, No. 4. pp. 1561-1566.
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