Partial RAG deficiency in humans induces dysregulated peripheral lymphocyte development and humoral tolerance defect with accumulation of T-bet(+) B cells

The recombination-activating genes (RAG) 1 and 2 are indispensable for diversifying the primary B cell receptor repertoire and pruning self-reactive clones via receptor editing in the bone marrow; however, the impact of RAG1/RAG2 on peripheral tolerance is unknown. Partial RAG deficiency (pRD) manif...

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Detalles Bibliográficos
Autores principales: Csomos, Krisztian, Ujhazi, Boglarka, Blazso, Peter, Herrera, Jose L., Tipton, Christopher M., Kawai, Tomoki, Gordon, Sumai, Ellison, Maryssa, Wu, Kevin, Stowell, Matthew, Haynes, Lauren, Cruz, Rachel, Zakota, Bence, Nguyen, Johnny, Altrich, Michelle, Geier, Christoph B., Sharapova, Svetlana, Dasso, Joseph F., Leiding, Jennifer W., Smith, Grace, Al-Herz, Waleed, de Barros Dorna, Mayra, Fadugba, Olajumoke, Fronkova, Eva, Kanderova, Veronika, Svaton, Michael, Henrickson, Sarah E., Hernandez, Joseph D., Kuijpers, Taco, Kandilarova, Snezhina Mihailova, Naumova, Elizaveta, Milota, Tomas, Sediva, Anna, Moshous, Despina, Neven, Benedicte, Saco, Tara, Sargur, Ravishankar, Savic, Sinisa, Sleasman, John, Sunkersett, Gauri, Ward, Brant R., Komatsu, Masanobu, Pittaluga, Stefania, Kumanovics, Attila, Butte, Manish J., Cancro, Michael P., Pillai, Shiv, Meffre, Eric, Notarangelo, Luigi D., Walter, Jolan E.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group US 2022
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9355881/
https://www.ncbi.nlm.nih.gov/pubmed/35902638
http://dx.doi.org/10.1038/s41590-022-01271-6

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