LYMPHOPROLIFERATIVE DISORDER AND IMBALANCED T-HELPER RESPONSE IN C/EBP-BETA-DEFICIENT MICE

被引:377
作者
SCREPANTI, I
ROMANI, L
MUSIANI, P
MODESTI, A
FATTORI, E
LAZZARO, D
SELLITTO, C
SCARPA, S
BELLAVIA, D
LATTANZIO, G
BISTONI, F
FRATI, L
CORTESE, R
GULINO, A
CILIBERTO, G
COSTANTINI, F
POLI, V
机构
[1] IST RIC BIOL MOLEC P ANGELETTI,ROME,ITALY
[2] UNIV ROMA LA SAPIENZA,DIPARTIMENTO MED SPERIMENTALE,I-00185 ROME,ITALY
[3] UNIV PERUGIA,DIPARTIMENTO MED SPERIMENTALE & SCI BIOCHIM,I-06100 PERUGIA,ITALY
[4] UNIV CHIETI,DIPARTIMENTO PATOL UMANA & MED SOCIALE,CHIETI,ITALY
[5] UNIV LAQUILA,DIPARTIMENTO MED SPERIMENTALE,I-67100 LAQUILA,ITALY
[6] COLUMBIA UNIV,DEPT GENET & DEV,NEW YORK,NY 10032
关键词
CASTELMANS DISEASE; C; EBP-BETA; IL-6; GENE TARGETING; T-HELPER;
D O I
10.1002/j.1460-2075.1995.tb07185.x
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
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.
引用
收藏
页码:1932 / 1941
页数:10
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