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Models to explore the molecular function and regulation of AIRE
Joint Authors
Lovewell, Thomas
Tazi Ahnini, Rashid
Source
The Egyptian Journal of Medical Human Genetics
Issue
Vol. 12, Issue 2 (31 Dec. 2011), pp.107-115, 9 p.
Publisher
Egyptian Society of Human Genetics
Publication Date
2011-12-31
Country of Publication
Egypt
No. of Pages
9
Main Subjects
Topics
Abstract EN
Mutations in the Autoimmune Regulator (AIRE) gene are responsible for Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy (APECED).
Within the thymic medulla, AIRE regulates the expression of a large number of tissue-specific self-antigens (TSAs) and the recognisation of these TSAs by auto-reactive T-cells is a prerequisite step for thymic negative selection.
APECED patients will therefore develop multi-organ autoimmune disease because of the defective role of AIRE in thymic negative selection.
Aire-deficient mice also develop multi-organ autoimmune disease and in this review we will focus on how both animal and cellular models have been used to dissect biochemical function of AIRE/Aire which is an essential step toward the understanding disease pathogenesis.
American Psychological Association (APA)
Lovewell, Thomas& Tazi Ahnini, Rashid. 2011. Models to explore the molecular function and regulation of AIRE. The Egyptian Journal of Medical Human Genetics،Vol. 12, no. 2, pp.107-115.
https://search.emarefa.net/detail/BIM-380287
Modern Language Association (MLA)
Lovewell, Thomas& Tazi Ahnini, Rashid. Models to explore the molecular function and regulation of AIRE. The Egyptian Journal of Medical Human Genetics Vol. 12, no. 2 (2011), pp.107-115.
https://search.emarefa.net/detail/BIM-380287
American Medical Association (AMA)
Lovewell, Thomas& Tazi Ahnini, Rashid. Models to explore the molecular function and regulation of AIRE. The Egyptian Journal of Medical Human Genetics. 2011. Vol. 12, no. 2, pp.107-115.
https://search.emarefa.net/detail/BIM-380287
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references : p. 113-115
Record ID
BIM-380287