TY - JOUR
T1 - Inactivation of the IL-6 gene prevents development of multicentric Castleman's disease in C/EBPβ-deficient mice
AU - Screpanti, Isabella
AU - Musiani, Piero
AU - Bellavia, Diana
AU - Cappelletti, Manuela
AU - Aiello, Francesca B.
AU - Maroder, Marella
AU - Frati, Luigi
AU - Modesti, Andrea
AU - Gulino, Alberto
AU - Poli, Valeria
PY - 1996/10/1
Y1 - 1996/10/1
N2 - 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.
AB - 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.
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U2 - 10.1084/jem.184.4.1561
DO - 10.1084/jem.184.4.1561
M3 - Article
C2 - 8879230
AN - SCOPUS:10244246599
VL - 184
SP - 1561
EP - 1566
JO - Journal of Experimental Medicine
JF - Journal of Experimental Medicine
SN - 0022-1007
IS - 4
ER -