Sonic Hedgehog regulates thymic epithelial cell differentiation
José Ignacio Saldaña,Anisha Solanki,Ching-In Lau,Hemant Sahni,Susan Ross,Anna L. Furmanski,Masahiro Ono,Georg A. Holländer,Tessa Crompton +8 more
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TLDR
Treatment of human thymus explants with recombinant Shh or neutralising anti-Shh antibody indicated that the Hedgehog pathway is also involved in regulation of differentiation from DP to mature SP T cells in the humanThymus.About:
This article is published in Journal of Autoimmunity.The article was published on 2016-04-01 and is currently open access. It has received 34 citations till now. The article focuses on the topics: TEC & Sonic hedgehog.read more
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Journal ArticleDOI
Thymic Epithelial Cells
Jakub Abramson,Graham Anderson +1 more
TL;DR: Both historical and the most recent advances in the understanding of the cellular and molecular mechanisms controlling TEC development, function, dysfunction, and regeneration are reviewed.
Journal ArticleDOI
EXTL3 mutations cause skeletal dysplasia, immune deficiency, and developmental delay
Stefano Volpi,Yasuhiro Yamazaki,Patrick M. Brauer,Ellen van Rooijen,Atsuko Hayashida,Anne Slavotinek,Hye Sun Kuehn,Maja Di Rocco,Carlo Rivolta,Ileana Bortolomai,Likun Du,Kerstin Felgentreff,Lisa Ott de Bruin,Kazutaka Hayashida,George Freedman,Genni Enza Marcovecchio,Kelly Capuder,Prisni Rath,Nicole Luche,Elliott J. Hagedorn,Antonella Buoncompagni,Beryl Royer-Bertrand,Silvia Giliani,Pietro Luigi Poliani,Luisa Imberti,Kerry Dobbs,Fabienne E. Poulain,Alberto Martini,John P. Manis,Robert J. Linhardt,Marita Bosticardo,Sergio D. Rosenzweig,Hane Lee,Jennifer M. Puck,Juan Carlos Zúñiga-Pflücker,Leonard I. Zon,Pyong Woo Park,Andrea Superti-Furga,Luigi D. Notarangelo +38 more
TL;DR: Data identify EXTL3 mutations as a novel cause of severe immune deficiency with skeletal dysplasia and developmental delay and underline a crucial role of HS in thymopoiesis and skeletal and brain development.
Journal ArticleDOI
Thymic Epithelial Cells Contribute to Thymopoiesis and T Cell Development.
Hongxia Wang,Wenrong Pan,Lei Zheng,Xiao-Ping Zhong,Liang Tan,Zhanfeng Liang,Jing He,Pingfeng Feng,Yong Zhao,Yu-Rong Qiu +9 more
TL;DR: Recent advances in classification, development, and maintenance of TECs and mechanisms that control TEC functions during thymic involution and central tolerance are discussed.
Journal ArticleDOI
Common Variable Immunodeficiency and Liver Involvement
Junmin Song,Junmin Song,Ana Lleo,Guo Xiang Yang,Weici Zhang,Christopher L. Bowlus,M. Eric Gershwin,Patrick S.C. Leung +7 more
TL;DR: Interestingly, autoimmunity can be the only clinical manifestation of CVID at the time of diagnosis and may even develop prior to hypogammaglobulinemia, and further understanding in the etiology and pathophysiology will facilitate early diagnosis and treatments to improve prognosis.
Journal ArticleDOI
Sonic Hedgehog signaling limits atopic dermatitis via Gli2-driven immune regulation
Eleftheria Papaioannou,Diana C. Yánez,Diana C. Yánez,Susan Ross,Ching-In Lau,Anisha Solanki,Mira Manilal Chawda,Alex Virasami,Ismael Ranz,Masahiro Ono,Masahiro Ono,Ryan F.L. O'Shaughnessy,Ryan F.L. O'Shaughnessy,Tessa Crompton +13 more
TL;DR: An important role is established for Shh upregulation in preventing AD, by increased Gli-driven, Treg cell–mediated immune suppression, paving the way for a potential new therapeutic strategy.
References
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Journal ArticleDOI
Cyclopia and defective axial patterning in mice lacking Sonic hedgehog gene function.
Chin Chiang,Ying Litingtung,Eric Lee,Keith E. Young,Jeffrey L Corden,Heiner Westphal,Philip A. Beachy +6 more
TL;DR: Targeted gene disruption in the mouse shows that the Sonic hedgehog(Shh) gene plays a critical role in patterning of vertebrate embryonic tissues, including the brain and spinal cord, the axial skeleton and the limbs.
Journal ArticleDOI
Vertebrate Hedgehog signalling modulated by induction of a Hedgehog-binding protein
TL;DR: These findings support a model in which Hip attenuates Hedgehog signalling as a result of binding to Hedgehog proteins: a negative regulatory feedback loop established in this way could thus modulate the responses to any Hedgehog signal.
Journal ArticleDOI
CD5 Expression Is Developmentally Regulated By T Cell Receptor (TCR) Signals and TCR Avidity
TL;DR: The results demonstrate that low level expression of CD5 on CD4−CD8− (double negative, DN) thymocytes is independent of TCR gene rearrangement; however, induction ofCD5 surface expression on DN thymocyte requires engagement of the pre-TCR and is dependent upon the activity of p56lck.
Journal ArticleDOI
RANK signals from CD4+3− inducer cells regulate development of Aire-expressing epithelial cells in the thymic medulla
Simona W. Rossi,Mi-Yeon Kim,Andreas Leibbrandt,Sonia M. Parnell,William E. Jenkinson,Stephanie H. Glanville,Fiona M. McConnell,Hamish S. Scott,Josef M. Penninger,Eric J. Jenkinson,Peter J. L. Lane,Graham Anderson +11 more
TL;DR: A novel role for a CD4+3− inducer cell population, previously linked to development of organized secondary lymphoid structures and maintenance of T cell memory in the functional regulation of Aire-mediated promiscuous gene expression in the thymus, is demonstrated.
Journal ArticleDOI
Gene regulatory logic for reading the Sonic Hedgehog signaling gradient in the vertebrate neural tube.
Nikolaos Balaskas,Ana Ribeiro,Jasmina Panovska,Eric Dessaud,Noriaki Sasai,Karen M. Page,James Briscoe,Vanessa Ribes +7 more
TL;DR: It is shown that a spatially and temporally changing gradient of Shh signaling is interpreted by the regulatory logic of a downstream transcriptional network that provides robustness and reliability to tissue patterning.