Recombinase activating gene enzymes of lymphocytes

Luigi D. Notarangelo, Sandro Santagata, Anna Villa

Research output: Contribution to journalArticle

Abstract

Expression of T-cell receptor and surface immunoglobulins on T and B lymphocytes, respectively, is strictly dependent on the variable, (diversity) joining exon (V(D)J) recombination process, which is initiated by the lymphoid-specific recombinase activating gene proteins 1 and 2 (RAG1 and RAG2). Recent advances have highlighted the functional organization of the RAG1 and RAG2 proteins and have provided important information on the regulation of RAG gene expression. Depending on the severity of their effects on the V(D)J recombination process, mutations of the RAG genes account for a spectrum of combined immune deficiencies in humans.

Original languageEnglish
Pages (from-to)41-46
Number of pages6
JournalCurrent Opinion in Hematology
Volume8
Issue number1
DOIs
Publication statusPublished - 2001

ASJC Scopus subject areas

  • Hematology

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