Utilize este identificador para referenciar este registo: http://hdl.handle.net/10451/15877
Título: Human FOXN1-deficiency is associated with αβ double-negative and FoxP3+ T-cell expansions that are distinctly modulated upon thymic transplantation.
Autor: Albuquerque, A. S.
Marques, J. G.
Silva, S. L.
Ligeiro, D.
Devlin, B. H.
Dutrieux, J.
Cheynier, R.
Pignata, C.
Victorino, R. M.
Markert, M. L.
Sousa, A. E.
Data: 2002
Editora: Public Library of Science
Citação: PLoS ONE May 2012 | Volume 7 | Issue 5 | e37042
Resumo: Forkhead box N1 (FOXN1) is a transcription factor crucial for thymic epithelium development and prevention of its involution. Investigation of a patient with a rare homozygous FOXN1 mutation (R255X), leading to alopecia universalis and thymus aplasia, unexpectedly revealed non-maternal circulating T-cells, and, strikingly, large numbers of aberrant doublenegative ab T-cells (CD4negCD8neg, DN) and regulatory-like T-cells. These data raise the possibility that a thymic rudiment persisted, allowing T-cell development, albeit with disturbances in positive/negative selection, as suggested by DN and FoxP3+ cell expansions. Although regulatory-like T-cell numbers normalized following HLA-mismatched thymic transplantation, the abDN subset persisted 5 years post-transplantation. Involution of thymus allograft likely occurred 3 years post-transplantation based on sj/bTREC ratio, which estimates intrathymic precursor T-cell divisions and, consequently, thymic explant output. Nevertheless, functional immune-competence was sustained, providing new insights for the design of immunological reconstitution strategies based on thymic transplantation, with potential applications in other clinical settings.
Descrição: © 2012 Albuquerque et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Peer review: yes
URI: http://dx.doi.org/ 10.1371/journal.pone.0037042
ISSN: 1932-6203
Aparece nas colecções:IMM - Artigos em Revistas Internacionais
FM-LIC - Artigos em Revistas Internacionais

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