Immune markers in the RASopathy neurofibromatosis type 1

dc.creatorNavarro, Karen Cecília de Lima Torres
dc.creatorLima, Giselle
dc.creatorSilva, Ana Ana Cristina Simões e
dc.creatorLubambo, Isabela
dc.creatorRodrigues, Luiz O.
dc.creatorRodrigues, Luiza
dc.creatorSilveira, Kátia D.
dc.creatorVieira, Érica Leandro Marciano
dc.creatorSilva, Marco Aurelio Romano
dc.creatorMiranda, Débora Marques de
dc.date2017-04-12T18:56:48Z
dc.date2017-04-12T18:56:48Z
dc.date2016
dc.date.accessioned2023-09-27T00:06:58Z
dc.date.available2023-09-27T00:06:58Z
dc.identifierNAVARRO, Karen Cecília de Lima Torres et al. Immune markers in the RASopathy neurofibromatosis type 1. J Neuroimmunol, v. 295-296, p. 122-129, 2016.
dc.identifier0165-5728
dc.identifierhttps://www.arca.fiocruz.br/handle/icict/18432
dc.identifier10.1016/j.jneuroim.2016.04.008
dc.identifier.urihttps://repositorioslatinoamericanos.uchile.cl/handle/2250/8897592
dc.descriptionNeurofibromatosis type 1 (NF1) is a genetic disorder with an early mortality determined mostly by malignancy. Little is known about the immunosurveillance factors in NF1 patients. In this study we evaluated inflammatory markers and their cellular sources in NF1 patients to try understanding the relation of immune factors and the tumorigenesis that characterizes the disease. Using flow cytometry and ELISA, we assayed cytokines, co-stimulatory molecules, the functional state of circulating blood cells and cytokine plasma levels in a case-control transversal study. The frequency of CD4+ T cells seems reduced. In addition, a shift towards an anti-inflammatory profile was observed in cells expressing cytokines, except for a small subpopulation of CD8+ T cells that displayed an increased frequency of cells expressing the pro-inflammatory cytokine Tumor necrosis factor (TNF-α), while plasma soluble levels of Transforming growth factor-beta (TGF-β) and interleukin-6 (IL-6) were increased in NF1 patients. Knowledge of the regulation of NF1 and the role of TGF-beta signaling pathway in malignant peripheral nerve sheath tumor pathogenesis might shed light on molecular carcinogenesis mechanisms and lead to putative interventions both in prevention and treatment of malignant tumors.
dc.description2025-01-01
dc.formatapplication/pdf
dc.languageeng
dc.publisherElsevier/North-Holland
dc.rightsrestricted access
dc.subjectneurofibromatose
dc.subjectCarcinogenesis
dc.subjectCytokines
dc.subjectLymphocytes
dc.subjectMonocytes
dc.subjectNeurofibromatosis type 1
dc.titleImmune markers in the RASopathy neurofibromatosis type 1
dc.typeArticle


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