Journal
JOURNAL OF CLINICAL INVESTIGATION
Volume 105, Issue 9, Pages 1233-1241Publisher
AMER SOC CLINICAL INVESTIGATION INC
DOI: 10.1172/JCI7610
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- NINDS NIH HHS [NS28840, R01 NS028840] Funding Source: Medline
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We have found that EGF-R expression is associated with the development of the Schwann cell-derived rumors characteristic of neurofibromatosis type 1 (NF1) and in animal models of this disease. This is surprising, because Schwann cells normally lack EGF-R and respond to ligands other than EGF. Nevertheless, immunoblotting, Northern analysis, and immunohistochemistry revealed that each of 3 malignant peripheral nerve sheath tumor (MPNST) cell lines from NF1 patients expressed the EGFR, as did 7 of 7 other primary MPNSTs, a non-NF 1 MPNST cell line, and the S100(+) cells from each of 9 benign neurofibromas. Furthermore, transformed derivatives of Schwann cells from NF1 (-/-) mouse embryos also expressed the EGF-R. All of the cells or cell lines expressing EGF-R responded to EGF by activation of downstream signaling pathways. Thus, EGF-R expression may play an important role in NF1 tumorigenesis and Schwann cell transformation. Consistent with this hypothesis, growth of NF1 MPNST lines and the transformed NF1 (-/-) mouse embryo Schwann cells was greatly stimulated by EGF in vitro and could be blocked by agents that antagonize EGF-R function.
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