Thymic stromal defects and immune dysregulation in hypomorphic Rag deficiency: implications for hematopoietic cell transplantation.
BACKGROUND Thymic epithelial cells (TEC) are essential for positive and negative selection during T-cell development. Hypomorphic RAG deficiency is characterized by T-cell lymphopenia and an increased risk of infections and autoimmunity. However, whether abnormalities of TEC composition and function also contribute to...