Abstrakt: |
A recent report from the Children's Hospital of Philadelphia discusses primary immune regulatory disorders (PIRDs), a subset of inborn errors of immunity characterized by immune dysregulation. The researchers focused on PIRDs that amplify T cell receptor signaling, leading to chronic inflammation and antigen presentation similar to chronic infections. They found that these PIRDs exhibit a T cell exhaustion-like process, characterized by increased inhibitory receptor expression, altered transcriptional networks, and impaired T cell functions. The study aims to identify shared patterns of CD8 T cell dysfunction in these rare disorders to develop novel therapeutic strategies and enhance understanding of CD8 T cell function. [Extracted from the article] |