期刊
NEUROBIOLOGY OF DISEASE
卷 29, 期 2, 页码 278-292出版社
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.nbd.2007.09.002
关键词
merlin; NF2; neurofibromatosis 2; meningioma; arachnoidal; senescence; actin cytoskeleton; alpha-catenin; contact inhibition; RNA interference
资金
- NINDS NIH HHS [NS041917, P30 NS045776-05, P01 NS024279, R01 NS041917-04, P30 NS045776-02, P01 NS024279-160010, P30 NS045776-019003, P30 NS045776-01, NS024279, P01 NS024279-120001, P30 NS045776, R01 NS041917-03, P30 NS045776-03, P01 NS024279-140001, R01 NS041917-01, P01 NS024279-15A10010, P01 NS024279-130001, P30 NS045776-019001, P30 NS045776-04, R01 NS041917, NS045776, R01 NS041917-05, R01 NS041917-02] Funding Source: Medline
Meningiomas, common tumors arising from arachnoidal cells of the meninges, may occur sporadically, or in association with the inherited disorder, neurofibromatosis 2 (NF2). Most sporadic meningiomas result from NF2 inactivation, resulting in loss of tumor suppressor merlin, implicated in regulating membrane-cytoskeletal organization. To investigate merlin function in an authentic target cell type for NF2 tumor formation, we established primary cultures from genetically-matched meningioma and normal arachnoidal tissues. Our studies revealed novel and distinct cell biological and biochemical properties unique to merlin-deficient meningioma cells compared to merlin-expressing arachnoidal and meningioma cells, and other NF2-deficient cell types. Merlin-deficient meningioma cells displayed cytoskeletal and cell contact defects, altered cell morphology and growth properties, most notably cell senescence, implicating the activation of senescence pathways in limiting benign meningioma growth. Merlin suppression by RNAi in arachnoidal cells replicated merlin-deficient meningioma features, thus establishing these cell systems as disease-relevant models for studying NF2 tumorigenesis. (C) 2007 Elsevier Inc. All rights reserved.
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