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Engrafted maternal T cells in a severe combined immunodeficiency patient
express T-cell receptor variable beta segments characterized by a
restricted V-D-J junctional diversity
A Sottini, E Quiros-Roldan, LD Notarangelo, A Malagoli, D Primi and L Imberti
Consorzio per le Biotecnologie, Terzo Laboratorio Analisi, Servizio di
Immunologia Clinica, Spedali Civili, Brescia, Italy.
To better understand the peculiar functional behavior of engrafted maternal
T cells in a severe combined immunodeficiency (SCID) patient, we
characterized, at the molecular level, the T-cell repertoire of a SCID
child with a high number of engrafted, mature, activated lymphocytes. We
found that, although these transplacentally acquired T cells express a
random set of T-cell receptor variable beta (TCRBV) segments, the TCRBV
transcripts are characterized by an extremely restricted V-D-J junctional
diversity. Only a few cDNA clones were dominant among the TCRBV4+, TCRBV6+,
and TCRBV20+ populations in engrafted cells, whereas the same TCRBV chains
expressed by the mother's lymphocytes had the expected junctional
hetero-geneity. Highly diverse and polyclonal junctions were also expressed
by maternal cells activated in mixed lymphocyte reaction by Epstein-Barr
virus (EBV)- transformed B lymphocytes from the patient, indicating that
the strong clonal selection that characterizes the engrafted cells
repertoire is probably not due to allorecognition. Furthermore, we report
that the repertoire of the transplacentally acquired lymphocytes is dynamic
over time and is characterized by waves of expression and contraction of
selected clones, expressing different TCRBV segments. These results help to
explain some of the abnormal functional behaviors of engrafted maternal
cells and raise new questions regarding the mechanisms responsible for the
restricted clonal diversity.
Volume 85,
Issue 8,
pp. 2105-2113,
04/15/1995
Copyright © 1995 by The American Society of Hematology
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