title: Thymic epithelial cell development and its dysfunction in human diseases.
abstract: Thymic epithelial cells (TECs) are the key components in thymic microenvironment for T cells development. TECs, composed of cortical and medullary TECs, are derived from a common bipotent progenitor and undergo a stepwise development controlled by multiple levels of signals to be functionally mature for supporting thymocyte development. Tumor necrosis factor receptor (TNFR) family members including the receptor activator for NF κ B (RANK), CD40, and lymphotoxin β receptor (LT β R) cooperatively control the thymic medullary microenvironment and self-tolerance establishment. In addition, fibroblast growth factors (FGFs), Wnt, and Notch signals are essential for establishment of functional thymic microenvironment. Transcription factors Foxn1 and autoimmune regulator (Aire) are powerful modulators of TEC development, differentiation, and self-tolerance. Dysfunction in thymic microenvironment including defects of TEC and thymocyte development would cause physiological disorders such as tumor, infectious diseases, and autoimmune diseases. In the present review, we will summarize our current understanding on TEC development and the underlying molecular signals pathways and the involvement of thymus dysfunction in human diseases.
authors.affiliation: Transplantation Biology Research Division, State Key Laboratory of Biomembrane and Membrane Biotechnology, Institute of Zoology, Chinese Academy of Sciences, Beichen Xi Road 1-5, Chaoyang District, Beijing 100101, China.
authors.forename: Lina
authors.identifier: 0000-0002-4202-2713
authors.initials: L
authors.lastname: Sun
authors.affiliation: Transplantation Biology Research Division, State Key Laboratory of Biomembrane and Membrane Biotechnology, Institute of Zoology, Chinese Academy of Sciences, Beichen Xi Road 1-5, Chaoyang District, Beijing 100101, China.
authors.forename: Hongran
authors.identifier: 0000-0002-4591-512X
authors.initials: H
authors.lastname: Li
authors.affiliation: Transplantation Biology Research Division, State Key Laboratory of Biomembrane and Membrane Biotechnology, Institute of Zoology, Chinese Academy of Sciences, Beichen Xi Road 1-5, Chaoyang District, Beijing 100101, China.
authors.forename: Haiying
authors.initials: H
authors.lastname: Luo
authors.affiliation: Transplantation Biology Research Division, State Key Laboratory of Biomembrane and Membrane Biotechnology, Institute of Zoology, Chinese Academy of Sciences, Beichen Xi Road 1-5, Chaoyang District, Beijing 100101, China.
authors.forename: Yong
authors.initials: Y
authors.lastname: Zhao
mesh_terms: D004847:Epithelial Cells / Q000473:pathology*; D006801:Humans; D008206:Lymphatic Diseases / Q000473:pathology* / Q000503:physiopathology*; D038081:Organogenesis; D013950:Thymus Gland / Q000254:growth & development / Q000473:pathology* / Q000503:physiopathology*