From Murine to Human NudeSCID: The Thymus, T-Cell Development and the Missing Link
Joint Authors
Giardino, Giuliana
Pignata, Claudio
Vigliano, Ilaria
Romano, Rosa
Fusco, Anna
Iannace, Leucio
Palamaro, Loredana
Maio, Stefano
Source
Journal of Immunology Research
Issue
Vol. 2012, Issue 2012 (31 Dec. 2011), pp.1-12, 12 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2012-01-16
Country of Publication
Egypt
No. of Pages
12
Main Subjects
Abstract EN
Primary immunodeficiencies (PIDs) are disorders of the immune system, which lead to increased susceptibility to infections.
T-cell defects, which may affect T-cell development/function, are approximately 11% of reported PIDs.
The pathogenic mechanisms are related to molecular alterations not only of genes selectively expressed in hematopoietic cells but also of the stromal component of the thymus that represents the primary lymphoid organ for T-cell differentiation.
With this regard, the prototype of athymic disorders due to abnormal stroma is the Nude/SCID syndrome, first described in mice in 1966.
In man, the DiGeorge Syndrome (DGS) has long been considered the human prototype of a severe T-cell differentiation defect.
More recently, the human equivalent of the murine Nude/SCID has been described, contributing to unravel important issues of the T-cell ontogeny in humans.
Both mice and human diseases are due to alterations of the FOXN1, a developmentally regulated transcription factor selectively expressed in skin and thymic epithelia.
American Psychological Association (APA)
Romano, Rosa& Palamaro, Loredana& Fusco, Anna& Iannace, Leucio& Maio, Stefano& Vigliano, Ilaria…[et al.]. 2012. From Murine to Human NudeSCID: The Thymus, T-Cell Development and the Missing Link. Journal of Immunology Research،Vol. 2012, no. 2012, pp.1-12.
https://search.emarefa.net/detail/BIM-994436
Modern Language Association (MLA)
Romano, Rosa…[et al.]. From Murine to Human NudeSCID: The Thymus, T-Cell Development and the Missing Link. Journal of Immunology Research Vol. 2012, no. 2012 (2011), pp.1-12.
https://search.emarefa.net/detail/BIM-994436
American Medical Association (AMA)
Romano, Rosa& Palamaro, Loredana& Fusco, Anna& Iannace, Leucio& Maio, Stefano& Vigliano, Ilaria…[et al.]. From Murine to Human NudeSCID: The Thymus, T-Cell Development and the Missing Link. Journal of Immunology Research. 2012. Vol. 2012, no. 2012, pp.1-12.
https://search.emarefa.net/detail/BIM-994436
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-994436