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Expression of SH2D1A in five classical Hodgkin's disease‐derived cell lines
Author(s) -
Kis Loránd L.,
Nagy Noémi,
Klein George,
Klein Eva
Publication year - 2003
Publication title -
international journal of cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.475
H-Index - 234
eISSN - 1097-0215
pISSN - 0020-7136
DOI - 10.1002/ijc.10986
Subject(s) - lymphoma , biology , virus , cancer research , phenotype , immunology , mononucleosis , epstein–barr virus , virology , gene , genetics
The Src homology 2 domain protein 1A (SH2D1A) is a small, 128‐amino acid protein consisting of a single SH2 domain; it is probably involved in signal regulation. It is expressed in activated T and natural killer (NK) cells, but not in B lymphocytes. It was discovered in studies on the rare hereditary condition X‐linked lymphoproliferative disease (XLP). Individuals with this condition either lack or carry an altered protein. The serious symptoms (fatal mononucleosis) present almost exclusively at the first encounter with Epstein‐Barr virus (EBV). The absence of SH2D1A in B cells, which are the targets of EBV, has to be reconciled with this clinical situation. In an earlier search for B lymphocytes expressing SH2D1A, we detected it in EBV‐carrying type I Burkitt's lymphoma (BL) lines. We now show SH2D1A in 5 EBV‐negative classical Hodgkin's disease (HD)‐derived cell lines. Two lines belong to the T lineage and 3 to the B lineage. One B‐HD line, which originated from nodular lymphocyte‐predominant Hodgkin's lymphoma and differed in phenotype, was SH2D1A‐negative. This finding is in accordance with the previously reported abundant SH2D1A mRNA in Hodgkin and Reed‐Sternberg (HRS) cells. We thus found SH2D1A expression in lines of malignant origin assigned to the B lineage. Its presence in HRS cells may lead us closer to an understanding of the pathophysiology of the serious syndrome connected with EBV infection in XLP patients, because HRS‐like cells have been detected in the lymphoid tissue of patients with infectious mononucleosis. It is likely therefore that in addition to the demonstrated functional defect of T and NK cells imposed by the SH2D1A mutation, the behavior of certain EBV‐infected B lymphocytes is also modified. © 2003 Wiley‐Liss, Inc.