Lymphoproliferative disorder and imbalanced T‐helper response in C/EBP beta‐deficient mice

C/EBP beta 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/EBP beta develop a pathology similar to mice overexpressing IL‐6 and nearly identical to multicentric Castleman...

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Bibliographic Details
Published in:The EMBO journal 1995-05, Vol.14 (9), p.1932-1941
Main Authors: Screpanti, I., Romani, L., Musiani, P., Modesti, A., Fattori, E., Lazzaro, D., Sellitto, C., Scarpa, S., Bellavia, D., Lattanzio, G.
Format: Article
Language:English
Subjects:
Animals
Antibodies, Fungal - biosynthesis
Candidiasis - etiology
Castleman Disease - etiology
Castleman Disease - genetics
Castleman Disease - immunology
CCAAT-Enhancer-Binding Proteins
CD4-Positive T-Lymphocytes - immunology
Disease Models, Animal
DNA-Binding Proteins - genetics
DNA-Binding Proteins - immunology
Female
Humans
Interleukin-6 - blood
Interleukin-6 - genetics
Lymph Nodes - immunology
Lymph Nodes - pathology
Lymphoproliferative Disorders - etiology
Lymphoproliferative Disorders - genetics
Lymphoproliferative Disorders - immunology
Macrophage Activation
Male
Mice
Mice, Mutant Strains
Myeloproliferative Disorders - etiology
Myeloproliferative Disorders - genetics
Myeloproliferative Disorders - immunology
Nitric Oxide - biosynthesis
Nuclear Proteins - genetics
Nuclear Proteins - immunology
Spleen - immunology
Spleen - pathology
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Summary:C/EBP beta 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/EBP beta 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/EBP beta is crucial for the correct functional regulation and homeostatic control of haemopoietic and lymphoid compartments.
ISSN:0261-4189
1460-2075
DOI:10.1002/j.1460-2075.1995.tb07185.x