Thymic Epithelial Cell Alterations and Defective Thymopoiesis Lead to Central and Peripheral Tolerance Perturbation in MHCII Deficiency

Major Histocompatibility Complex (MHC) class II (MHCII) deficiency (MHCII-D), also known as Bare Lymphocyte Syndrome (BLS), is a rare combined immunodeficiency due to mutations in genes regulating expression of MHCII molecules. MHCII deficiency results in impaired cellular and humoral immune respons...

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Detalles Bibliográficos
Autores principales: Ferrua, Francesca, Bortolomai, Ileana, Fontana, Elena, Di Silvestre, Dario, Rigoni, Rosita, Marcovecchio, Genni Enza, Draghici, Elena, Brambilla, Francesca, Castiello, Maria Carmina, Delfanti, Gloria, Moshous, Despina, Picard, Capucine, Taghon, Tom, Bordon, Victoria, Schulz, Ansgar S., Schuetz, Catharina, Giliani, Silvia, Soresina, Annarosa, Gennery, Andrew R., Signa, Sara, Dávila Saldaña, Blachy J., Delmonte, Ottavia M., Notarangelo, Luigi D., Roifman, Chaim M., Poliani, Pietro Luigi, Uva, Paolo, Mauri, Pier Luigi, Villa, Anna, Bosticardo, Marita
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2021
Materias:
Immunology
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8239840/
https://www.ncbi.nlm.nih.gov/pubmed/34211466
http://dx.doi.org/10.3389/fimmu.2021.669943

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