Interleukin-7 receptor α (IL-7Rα) deficiency: Cellular and molecular bases. Analysis of clinical, immunological, and molecular features in 16 novel patients

Silvia Giliani; Luigi Mori; Geneviève De Saint Basile; Francoise Le Deist; Carmen Rodriguez-Perez; Concetta Forino; Evelina Mazzolari; Sophie Dupuis; Ronit Elhasid; Aharon Kessel; Claire Galambrun; Juana Gil; Alain Fischer; Amos Etzioni; Luigi D. Notarangelo

doi:10.1111/j.0105-2896.2005.00234.x

Interleukin-7 receptor α (IL-7Rα) deficiency: Cellular and molecular bases. Analysis of clinical, immunological, and molecular features in 16 novel patients

Silvia Giliani^*, Luigi Mori, Geneviève De Saint Basile, Francoise Le Deist, Carmen Rodriguez-Perez, Concetta Forino, Evelina Mazzolari, Sophie Dupuis, Ronit Elhasid, Aharon Kessel, Claire Galambrun, Juana Gil, Alain Fischer, Amos Etzioni, Luigi D. Notarangelo

^*Corresponding author for this work

Research output: Contribution to journal › Review article › peer-review

160 Scopus citations

Abstract

Analysis of gene-targeted mice and patients with severe combined immunodeficiency due to mutations of the α chain of the interleukin-7 receptor (IL-7Rα) has shown important differences between mice and humans in the role played by IL-7 in lymphoid development. More recently, it has been shown that IL-7Rα is also shared by the receptor for another cytokine, thymic stromal lymphopoietin (TSLP). In this review, we discuss recent advances in IL-7- and TSLP-mediated signaling. We also report on the clinical and immunological features of 16 novel patients with IL-7Rα deficiency and discuss the results of hematopoietic stem cell transplantation.

Original language	English
Pages (from-to)	110-126
Number of pages	17
Journal	Immunological Reviews
Volume	203
DOIs	https://doi.org/10.1111/j.0105-2896.2005.00234.x
State	Published - Feb 2005
Externally published	Yes

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Giliani, S., Mori, L., De Saint Basile, G., Le Deist, F., Rodriguez-Perez, C., Forino, C., Mazzolari, E., Dupuis, S., Elhasid, R., Kessel, A., Galambrun, C., Gil, J., Fischer, A., Etzioni, A., & Notarangelo, L. D. (2005). Interleukin-7 receptor α (IL-7Rα) deficiency: Cellular and molecular bases. Analysis of clinical, immunological, and molecular features in 16 novel patients. Immunological Reviews, 203, 110-126. https://doi.org/10.1111/j.0105-2896.2005.00234.x

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abstract = "Analysis of gene-targeted mice and patients with severe combined immunodeficiency due to mutations of the α chain of the interleukin-7 receptor (IL-7Rα) has shown important differences between mice and humans in the role played by IL-7 in lymphoid development. More recently, it has been shown that IL-7Rα is also shared by the receptor for another cytokine, thymic stromal lymphopoietin (TSLP). In this review, we discuss recent advances in IL-7- and TSLP-mediated signaling. We also report on the clinical and immunological features of 16 novel patients with IL-7Rα deficiency and discuss the results of hematopoietic stem cell transplantation.",

author = "Silvia Giliani and Luigi Mori and {De Saint Basile}, Genevi{\`e}ve and {Le Deist}, Francoise and Carmen Rodriguez-Perez and Concetta Forino and Evelina Mazzolari and Sophie Dupuis and Ronit Elhasid and Aharon Kessel and Claire Galambrun and Juana Gil and Alain Fischer and Amos Etzioni and Notarangelo, {Luigi D.}",

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doi = "10.1111/j.0105-2896.2005.00234.x",

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Giliani, S, Mori, L, De Saint Basile, G, Le Deist, F, Rodriguez-Perez, C, Forino, C, Mazzolari, E, Dupuis, S, Elhasid, R, Kessel, A, Galambrun, C, Gil, J, Fischer, A, Etzioni, A & Notarangelo, LD 2005, 'Interleukin-7 receptor α (IL-7Rα) deficiency: Cellular and molecular bases. Analysis of clinical, immunological, and molecular features in 16 novel patients', Immunological Reviews, vol. 203, pp. 110-126. https://doi.org/10.1111/j.0105-2896.2005.00234.x

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AU - Giliani, Silvia

AU - Mori, Luigi

AU - De Saint Basile, Geneviève

AU - Le Deist, Francoise

AU - Rodriguez-Perez, Carmen

AU - Forino, Concetta

AU - Mazzolari, Evelina

AU - Dupuis, Sophie

AU - Elhasid, Ronit

AU - Kessel, Aharon

AU - Galambrun, Claire

AU - Gil, Juana

AU - Fischer, Alain

AU - Etzioni, Amos

AU - Notarangelo, Luigi D.

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AB - Analysis of gene-targeted mice and patients with severe combined immunodeficiency due to mutations of the α chain of the interleukin-7 receptor (IL-7Rα) has shown important differences between mice and humans in the role played by IL-7 in lymphoid development. More recently, it has been shown that IL-7Rα is also shared by the receptor for another cytokine, thymic stromal lymphopoietin (TSLP). In this review, we discuss recent advances in IL-7- and TSLP-mediated signaling. We also report on the clinical and immunological features of 16 novel patients with IL-7Rα deficiency and discuss the results of hematopoietic stem cell transplantation.

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JO - Immunological Reviews

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ER -

Interleukin-7 receptor α (IL-7Rα) deficiency: Cellular and molecular bases. Analysis of clinical, immunological, and molecular features in 16 novel patients

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