Ral overactivation in malignant peripheral nerve sheath tumors

V Bodempudi, F Yamoutpoor, W Pan… - … and cellular biology, 2009 - Taylor & Francis
V Bodempudi, F Yamoutpoor, W Pan, AZ Dudek, T Esfandyari, M Piedra…
Molecular and cellular biology, 2009Taylor & Francis
Ras leads an important signaling pathway that is deregulated in neurofibromatosis type 1
and malignant peripheral nerve sheath tumor (MPNST). In this study, we show that
overactivation of Ras and many of its downstream effectors occurred in only a fraction of
MPNST cell lines. RalA, however, was overactivated in all MPNST cells and tumor samples
compared to nontransformed Schwann cells. Silencing Ral or inhibiting it with a dominant-
negative Ral (Ral S28N) caused a significant reduction in proliferation, invasiveness, and in …
Ras leads an important signaling pathway that is deregulated in neurofibromatosis type 1 and malignant peripheral nerve sheath tumor (MPNST). In this study, we show that overactivation of Ras and many of its downstream effectors occurred in only a fraction of MPNST cell lines. RalA, however, was overactivated in all MPNST cells and tumor samples compared to nontransformed Schwann cells. Silencing Ral or inhibiting it with a dominant-negative Ral (Ral S28N) caused a significant reduction in proliferation, invasiveness, and in vivo tumorigenicity of MPNST cells. Silencing Ral also reduced the expression of epithelial mesenchymal transition markers. Expression of the NF1-GTPase-related domain (NF1-GRD) diminished the levels of Ral activation, implicating a role for neurofibromin in regulating RalA activation. NF1-GRD treatment caused a significant decrease in proliferation, invasiveness, and cell cycle progression, but cell death increased. We propose Ral overactivation as a novel cell signaling abnormality in MPNST that leads to important biological outcomes with translational ramifications.
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