Malignant peripheral nerve sheath tumours (MPNSTs) commonly occur in patients with neurofibromatosis type 1 (NF1). To identify potential molecular drivers, Mo et al. analysed the gene expression signatures in MPNSTs that arose in Nf1-deficient mice. They found upregulation of CXCR4, which is a receptor for the CXCL12 chemokine. Similar signalling alterations were also found in human NF1-deficient MPNSTs. Crucially, knockdown or small-molecule inhibition of CXCR4 slowed the growth of MPNSTs in mice, highlighting the potential value of CXCR4 as a therapeutic target.
ORIGINAL RESEARCH PAPER
Mo, W. et al. CXCR4/CXCL12 mediate autocrine cell- cycle progression in NF1-associated malignant peripheral nerve sheath tumors. Cell 152, 1077–1090 (2013)
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Burgess, D. Chemokine underpinnings of a tumour. Nat Rev Cancer 13, 221 (2013). https://doi.org/10.1038/nrc3506
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DOI: https://doi.org/10.1038/nrc3506
