Lymphoproliferative disorder imbalanced T-helper response in C/EBPβ-deficient mice

Isabella Screpanti, Luigina Romani, Piero Musiani, Andrea Modesti, Elena Fattori, Domenico Lazzaro, Carolina Sellitto, Susanna Scarpa, Diana Bellavia, Giuseppe Lattanzio, Francesco Bistoni, Luigi Frati, Riccardo Cortese, Alberto Gulino, Gennaro Ciliberto, Frank Costantini, Valeria Poli

Research output: Contribution to journalArticlepeer-review


C/EBPβ is considered a key element of interleukin-6 (IL-6) signalling as well as an important transcriptional regulator of the IL-6 gene itself. We describe here how mice lacking C/EBPβ develop a pathology similar to mice overexpressing IL-6 and nearly identical to multicentric Castleman's disease in human patients, with marked splenomegaly, peripheral lymphadenopathy and enhanced haemopoiesis. Humoral, innate and cellular immunity are also profoundly distorted, as shown by the defective activation of splenic macrophages, the strong impairement of IL-12 production, the increased susceptibility to Candida albicans infection and the altered T-helper function. Our data show that C/EBPβ is crucial for the correct functional regulation and homeostatic control of haemopoietic and lymphoid compartments.

Original languageEnglish
Pages (from-to)1932-1941
Number of pages10
JournalEMBO Journal
Issue number9
Publication statusPublished - 1995


  • C/EBPβ
  • Castelman's disease
  • Gene targeting
  • Ll-6
  • T-helper

ASJC Scopus subject areas

  • Cell Biology
  • Genetics


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