16 | Chapter 2 Abstract This review explores the intricate regulatory mechanisms governing RAG1 and RAG2 expression and activity during the development of B cells, with a particular focus on their implications in the development of lymphoid malignancies such as B-cell acute lymphoblastic leukemia (B-ALL). We summarize current knowledge on B-cell development, the mechanism of V(D)J recombination, and the role of the RAG1 and RAG2 (RAG1/2) complex. We discuss the role of the DNA damage response (DDR) during V(D)J recombination, shedding light on its involvement in the regulation of the activity and expression of RAG1/2. Understanding the interplay between the various regulatory processes provides insights into the potential links between aberrant V(D)J recombination, dysregulation of RAG1/2, and the onset of lymphoid malignancies, contributing to our understanding of the molecular events underlying these diseases.
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