FOXN1 Deficiency: from the Discovery to Novel Therapeutic Approaches.
J Clin Immunol
; 37(8): 751-758, 2017 Nov.
Article
em En
| MEDLINE
| ID: mdl-28932937
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.
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Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Pele
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Timo
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Linfócitos T
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Imunodeficiência Combinada Severa
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Folículo Piloso
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Fatores de Transcrição Forkhead
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Unhas
Limite:
Animals
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Humans
Idioma:
En
Ano de publicação:
2017
Tipo de documento:
Article