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F Rieux-Laucat, P Bahadoran, N Brousse, F Selz, A Fischer, F Le Deist, J P De Villartay
J Clin Invest. 1998;
102(2):312
doi:10.1172/JCI332
Abstract |
Full text
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O
menn's syndrome is an inherited human combined immunodeficiency condition characterized by the presence of a large population of activated and tissue-infiltrating T cells. Analysis of the TCRB repertoire revealed a highly restricted TCRBV usage in three patients. More strikingly, T cell clones from the three patients expressed TCRB chains with VDJ junction similarities, suggesting a common antigenic specificity. Analysis of the TCRA repertoire in one patient also revealed a restricted TCRAV usage. Finally, analysis of the TCRBV repertoire of tissue-infiltrating T cells in one patient suggested nonrandom tissue migration. These results suggest that the oligoclonal expansion of T cells observed in Omenn's syndrome could be the consequence of autoimmune proliferation generated by a profound defect in lymphocyte development.
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(23)
| Title and authors |
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Year |
Omenn syndrome does not live by V(D)J recombination alone :
Veronica Marrella, Virginia Maina, Anna Villa
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Current Opinion in Allergy and Clinical Immunology
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2011 |
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2010 |
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Rook's Textbook of Dermatology
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2010 |
Homeostatic expansion of autoreactive immunoglobulin-secreting cells in the Rag2 mouse model of Omenn syndrome
B. Cassani, P. L. Poliani, V. Marrella, F. Schena, A. V. Sauer, M. Ravanini, D. Strina, C. E. Busse, S. Regenass, H. Wardemann, A. Martini, F. Facchetti, M. van der Burg, A. G. Rolink, P. Vezzoni, F. Grassi, E. Traggiai, A. Villa
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Journal of Experimental Medicine
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2010 |
Defect of regulatory T cells in patients with Omenn syndrome
Barbara Cassani, Pietro Luigi Poliani, Daniele Moratto, Cristina Sobacchi, Veronica Marrella, Laura Imperatori, Donatella Vairo, Alessandro Plebani, Silvia Giliani, Paolo Vezzoni, Fabio Facchetti, Fulvio Porta, Luigi D. Notarangelo, Anna Villa, Raffaele Badolato
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Journal of Allergy and Clinical Immunology
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2010 |
Secondary immunologic consequences in chromosome 22q11.2 deletion syndrome (DiGeorge syndrome/velocardiofacial syndrome)
R. Zemble, E. Luning Prak, K. McDonald, D. McDonald-McGinn, E. Zackai, K. Sullivan
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Clinical Immunology
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2010 |
Decrease of Skin Infiltrating and Circulating CCR10+ T Cells Coincides with Clinical Improvement after Topical Tacrolimus in Omenn Syndrome
Claudia M Faaij, Nicola E Annels, Geertje Ruigrok, Mirjam van der Burg, Lynne M Ball, Robbert G Bredius, Maarten J van Tol, Arjan C Lankester
|
J Investig Dermatol
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2009 |
Rapid full engraftment and successful immune reconstitution after allogeneic hematopoietic stem cell transplantation with reduced intensity conditioning in Omenn syndrome
Jolanta Gozdzik, Wojciech Czogala, Szymon Skoczen, Aleksandra Krasowska-Kwiecien, Oktawiusz Wiecha, Anna Mordel, Ewa Lesko, Marcin Majka, Danuta Kowalczyk, Marek Zembala
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Pediatric Transplantation
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2009 |
Lack of iNKT cells in patients with combined immune deficiency due to hypomorphic RAG mutations.
Ponpan Matangkasombut, Muriel Pichavant, Doris E Saez, Silvia Giliani, Evelina Mazzolari, Andrea Finocchi, Anna Villa, Cristina Sobacchi, Patricia Cortes, Dale T Umetsu, Luigi D Notarangelo
|
Blood
|
2008 |
Clinical Immunology
Françoise Le Deist, Alain Fischer
|
Clinical Immunology
|
2008 |
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