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Characterization of B-cell lines established from two X-linked severe
combined immunodeficiency patients: interleukin-15 binds to the B cells but
is not internalized efficiently
S Kumaki, HD Ochs, M Timour, K Schooley, M Ahdieh, H Hill, K Sugamura, D Anderson, Q Zhu and D Cosman
Department of Molecular Biology, Immunex Research and Development Corp.,
Seattle, WA 98101, USA.
X-linked severe combined immunodeficiency (XSCID) is characterized by
absent or profoundly reduced numbers of T cells and normal numbers of B
cells in the circulation. Affected patients have mutations of the
interleukin-2 (IL-2) receptor gamma chain gene. Using Epstein-Barr
virus-transformed B-lymphoblastoid cell lines (B-LCLs) established from two
unrelated XSCID patients, we could show that neither expressed the IL-2
receptor gamma chain on the cell surface. A novel cytokine IL-15, which has
biologic activities similar to those of IL-2, could bind to the XSCID
B-LCLs in the absence of the gamma chain, although both the beta and gamma
chains of the human IL-2 receptor were previously shown to be required for
IL-15 binding by transfected COS cells. Furthermore, a significant
reduction and delay of IL-15 internalization by B lymphoblasts from XSCID
patients was observed when compared with that of normal control B-LCLs.
These results show the existence of a novel IL-15-specific receptor
component that contributes to IL-15 binding but is insufficient for IL-15
internalization in the absence of the IL-2 receptor gamma chain.
Volume 86,
Issue 4,
pp. 1428-1436,
08/15/1995
Copyright © 1995 by The American Society of Hematology
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