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Mutations in Conserved Regions of the Predicted RAG2 Kelch Repeats Block Initiation of V(D)J Recombination and Result in Primary Immunodeficiencies

The V(D)J recombination reaction is composed of multiple nucleolytic processing steps mediated by the recombination-activating proteins RAG1 and RAG2. Sequence analysis has suggested that RAG2 contains six kelch repeat motifs that are predicted to form a six-bladed β-propeller structure, with the se...

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Auteurs principaux: Gomez, Carlos A., Ptaszek, Leon M., Villa, Anna, Bozzi, Fabio, Sobacchi, Cristina, Brooks, Edward G., Notarangelo, Luigi D., Spanopoulou, Eugenia, Pan, Z. Q., Vezzoni, Paolo, Cortes, Patricia, Santagata, Sandro
Format: Artigo
Langue:Inglês
Publié: American Society for Microbiology 2000
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Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC86034/
https://ncbi.nlm.nih.gov/pubmed/10891502
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