Utilize este identificador para referenciar este registo: http://hdl.handle.net/10451/18297
Título: Reduced BAFF-R and increased TACI expression in common variable immunodeficiency
Autor: Barbosa, R. R.
Silva, S. L.
Silva, S. P.
Melo, A. C.
Pereira-Santos, M. C.
Barata, J. T.
Hammarström, L.
Cascalho, M.
Sousa, A. E.
Palavras-chave: Bcells
Common variable immunodeficiency
Data: 2014
Editora: Springer Verlag
Citação: Journal of Clinical Immunology, Volume 34, Issue 5, pp 573-583
Resumo: Purpose B-cell survival and differentiation critically depend on the interaction of BAFF-R and TACI with their ligands, BAFF and APRIL. Mature B-cell defects lead to Common Variable Immunodeficiency (CVID), which is associated with elevated serum levels of BAFF and APRIL. Nevertheless, BAFF-R and TACI expression in CVID and their relationship with ligand availability remain poorly understood. Methods and Results We found that BAFF-R expression was dramatically reduced on B cells of CVID patients, relative to controls. BAFF-R levels inversely correlated with serum BAFF concentration both in CVID and healthy subjects. We also found that recombinant BAFF stimulation reduced BAFF-R expression on B cells without decreasing transcript levels. On the other hand, CVID subjects had increased TACI expression on B cells in direct association with serum BAFF but not APRIL levels. Moreover, splenomegaly was associated with higher TACI expression, suggesting that perturbations of TACI function may underlie lymphoproliferation in CVID. Conclusions Our results indicate that availability of BAFF determines BAFF-R and TACI expression on B cells, and that BAFF-R expression is controlled by BAFF binding. Identification of the factors governing BAFF-R and TACI is crucial to understanding CVID pathogenesis, and B-cell biology in general, as well as to explore their potential as therapeutic targets.
Descrição: © Springer Science+Business Media New York 2014
Peer review: yes
URI: http://link.springer.com/article/10.1007%2Fs10875-014-0047-y
http://dx.doi.org/ 10.1007/s10875-014-0047-y
ISSN: 0271-9142
Versão do Editor: The definitive version is available at http://link.springer.com/
Aparece nas colecções:FM - Artigos em Revistas Internacionais
IMM - Artigos em Revistas Internacionais

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