Thymic stromal alterations and genetic disorders of immune system

• Main Author: Claudio Pignata (auth)
• Other Authors: Ana E. Sousa (auth)
• Format: Electronic Book Chapter
• Language: English
• Published: Frontiers Media SA 2015
• Series: Frontiers Research Topics
• Subjects: Medicine; Central Tolerance; Rag defects; Combined immunodeficiency; DiGeorge Syndrome; Foxn1; medullary thymic epithelial cells; APECED
• Online Access: DOAB: download the publication; DOAB: description of the publication

The pathogenic mechanisms underlying primary T-cell disorders are mainly related to molecular alterations of genes whose expression is intrinsic to hematopoietic cells. However, since the differentiation process requires a crosstalk among thymocytes and the thymic microenvironment, molecular alterat...