edoc

Clearing the AIRE : on the pathophysiological basis of the autoimmune polyendocrinopathy syndrome type-1

Shikama, Noriko and Nusspaumer, Gretel and Holländer, Georg A.. (2009) Clearing the AIRE : on the pathophysiological basis of the autoimmune polyendocrinopathy syndrome type-1. Endocrinology and metabolism clinics of North America, Vol. 38, H. 2 , S. 273-288, vii.

Full text not available from this repository.

Official URL: http://edoc.unibas.ch/dok/A6007698

Downloads: Statistics Overview

Abstract

Autoimmune polyendocrine syndrome type-1 clinically manifests as the triad of hypoparathyroidism, primary adrenocortical insufficiency, and chronic mucocutaneous candidiasis. Mutations in the gene that encodes the autoimmune regulator protein, AIRE, have been identified as the cause of the autoimmune polyendocrine syndrome type-1. The loss of immunologic tolerance to tissue-restricted antigens consequent to an absence of AIRE expression in the thymus results in the thymic export of autoreactive T cells that initiate autoimmunity. In this article, we discuss the role of AIRE in autoimmune polyendocrine syndrome type-1 and identify issues that still need to be addressed to fully understand the molecular pathophysiology of this complex syndrome.
Faculties and Departments:03 Faculty of Medicine > Departement Biomedizin > Department of Biomedicine, University Children's Hospital > Pediatric Immunology (Holländer)
UniBasel Contributors:Holländer, Georg
Item Type:Article, refereed
Article Subtype:Further Journal Contribution
Bibsysno:Link to catalogue
Publisher:W. B. Saunders
ISSN:0889-8529
Note:Publication type according to Uni Basel Research Database: Journal item
Related URLs:
Identification Number:
Last Modified:18 Jul 2014 09:10
Deposited On:18 Jul 2014 09:10

Repository Staff Only: item control page