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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 J+Exp+Med
2017 ; 214
(1
): 73-89
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Inherited CD70 deficiency in humans reveals a critical role for the CD70-CD27
pathway in immunity to Epstein-Barr virus infection
#MMPMID28011863
Izawa K
; Martin E
; Soudais C
; Bruneau J
; Boutboul D
; Rodriguez R
; Lenoir C
; Hislop AD
; Besson C
; Touzot F
; Picard C
; Callebaut I
; de Villartay JP
; Moshous D
; Fischer A
; Latour S
J Exp Med
2017[Jan]; 214
(1
): 73-89
PMID28011863
show ga
Epstein-Barr virus (EBV) infection in humans is a major trigger of malignant and
nonmalignant B cell proliferations. CD27 is a co-stimulatory molecule of T cells,
and inherited CD27 deficiency is characterized by high susceptibility to EBV
infection, though the underlying pathological mechanisms have not yet been
identified. In this study, we report a patient suffering from recurrent
EBV-induced B cell proliferations including Hodgkin's lymphoma because of a
deficiency in CD70, the ligand of CD27. We show that EBV-specific T lymphocytes
did not expand properly when stimulated with CD70-deficient EBV-infected B cells,
whereas expression of CD70 in B cells restored expansion, indicating that CD70 on
B cells but not on T cells is required for efficient proliferation of T cells.
CD70 was found to be up-regulated on B cells when activated and during EBV
infection. The proliferation of T cells triggered by CD70-expressing B cells was
dependent on CD27 and CD3 on T cells. Importantly, CD27-deficient T cells failed
to proliferate when stimulated with CD70-expressing B cells. Thus, the CD70-CD27
pathway appears to be a crucial component of EBV-specific T cell immunity and
more generally for the immune surveillance of B cells and may be a target for
immunotherapy of B cell malignancies.