منابع مشابه
The AIRE -230Y Polymorphism Affects AIRE Transcriptional Activity: Potential Influence on AIRE Function in the Thymus
BACKGROUND The autoimmune regulator (AIRE) is expressed in the thymus, particularly in thymic medullary epithelial cells (mTECs), and is required for the ectopic expression of a diverse range of peripheral tissue antigens by mTECs, facilitating their ability to perform negative selection of auto-reactive immature T-cells. The expression profile of peripheral tissue antigens is affected not only...
متن کاملAIRE deficiency in thymus of 2 patients with Omenn syndrome.
Omenn syndrome is a severe primary immunodeficiency with putative autoimmune manifestations of the skin and gastrointestinal tract. The disease is caused by hypomorphic mutations in recombination-activating genes that impair but do not abolish the process of VDJ recombination, leading to the generation of autoreactive T cells with a highly restricted receptor repertoire. Loss of central toleran...
متن کاملProjection of an Immunological Self Shadow Within the Thymus by the Aire Protein
33. L. G. Brieba, R. Sousa, Biochemistry 40, 3882 (2001). 34. P. A. Bullough, F. M. Hughson, J. J. Skehel, D. C. Wiley, Nature 371, 37 (1994). 35. J. Kuriyan, M. O’Donnell, J. Mol. Biol. 234, 915 (1993). 36. V. A. Bloomfield, D. M. Crothers, I. Tinoco Jr., Nucleic Acids: Structures, Properties, and Functions (University Science Books, Sausalito, CA, 2000). 37. F. M. Richards, Annu. Rev. Biophys...
متن کاملProjection of an immunological self shadow within the thymus by the aire protein.
Humans expressing a defective form of the transcription factor AIRE (autoimmune regulator) develop multiorgan autoimmune disease. We used aire- deficient mice to test the hypothesis that this transcription factor regulates autoimmunity by promoting the ectopic expression of peripheral tissue- restricted antigens in medullary epithelial cells of the thymus. This hypothesis proved correct. The mu...
متن کاملDevelopment of autoimmunity against transcriptionally unrepressed target antigen in the thymus of Aire-deficient mice.
Autoimmune regulator (AIRE) gene mutation is responsible for the development of organ-specific autoimmune disease with monogenic autosomal recessive inheritance. Although Aire has been considered to regulate the elimination of autoreactive T cells through transcriptional control of tissue-specific Ags in thymic epithelial cells, other mechanisms of AIRE-dependent tolerance remain to be investig...
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ژورنال
عنوان ژورنال: Journal of Experimental Medicine
سال: 2007
ISSN: 1540-9538,0022-1007
DOI: 10.1084/jem.2046iti2