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FOXN1 Deficiency: from the Discovery to Novel Therapeutic Approaches.

Authors :
Gallo, Vera
Cirillo, Emilia
Giardino, Giuliana
Pignata, Claudio
Source :
Journal of Clinical Immunology; Nov2017, Vol. 37 Issue 8, p751-758, 8p
Publication Year :
2017

Abstract

Since the discovery of FOXN1 deficiency, the human counterpart of the nude mouse, a growing body of evidence investigating the role of FOXN1 in thymus and skin, has been published. FOXN1 has emerged as fundamental for thymus development, function, and homeostasis, representing the master regulator of thymic epithelial and T cell development. In the skin, it also plays a pivotal role in keratinocytes and hair follicle cell differentiation, although the underlying molecular mechanisms still remain to be fully elucidated. The nude severe combined immunodeficiency phenotype is indeed characterized by the clinical hallmarks of athymia with severe T cell immunodeficiency, congenital alopecia, and nail dystrophy. In this review, we summarize recent discoveries in the field and give interesting perspective about new and promising therapeutic approaches for disorders of immune system with athymia. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
02719142
Volume :
37
Issue :
8
Database :
Complementary Index
Journal :
Journal of Clinical Immunology
Publication Type :
Academic Journal
Accession number :
125950800
Full Text :
https://doi.org/10.1007/s10875-017-0445-z