Sara Ortega‐Bertran

Induced pluripotent stem cell–derived models of malignant nerve sheath tumor progression mimic glial to neuro-mesenchymal transition and uncover therapeutic opportunities

Itziar Uriarte-Arrazola, Miriam Magallón‐Lorenz, Juana Fernández‐Rodríguez, Jiajing Zhang et autres

Neurofibromatosis Type 1 (NF1) predisposes to peripheral nerve tumor development. The progression from a benign plexiform neurofibroma (PNF) towards a deadly malignant peripheral nerve sheath tumor (MPNST) is not completely understood but commonly involves the sequential loss of NF1, CDKN2A, and polycomb …

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Identification of Malignant Peripheral Nerve Sheath Tumor subtypes with distinct genomic identities

Miriam Magallón‐Lorenz, Juana Fernandez-Rodriguez, Helena Mazuelas, Itziar Uriarte-Arrázola et autres

ABSTRACT Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft-tissue sarcomas arising sporadically or in people with neurofibromatosis type 1 (NF1). Their marked heterogeneity challenges diagnosis and has hampered an integrative view of MPNST molecular pathogenesis. Here, a thorough whole-genome and transcriptome …

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Personalized medicine strategy for MPNSTs: using precision oncology on PDOX models to inform tumor boards

Sara Ortega‐Bertran, Juana Fernández‐Rodríguez, Miriam Magallón‐Lorenz, Edgar Creus‐Bachiller et autres

BACKGROUND: Malignant peripheral nerve sheath tumors (MPNSTs) are a heterogeneous group of aggressive soft tissue sarcomas with poor prognosis. Currently there is a lack of effective treatments for MPNSTs. Here, we propose a personalized medicine approach that integrates a precision oncology strategy …

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Suppl. Figure 6 from Endoglin, a Novel Biomarker and Therapeutical Target to Prevent Malignant Peripheral Nerve Sheath Tumor Growth and Metastasis

Teresa González-Muñoz, Angela Di Giannatale, Susana Garcı́a-Silva, Vanesa Santos et autres

Dual inhibition of ENG and MEK efficiently blocks ENG-Smad1/5 and MAPK/ERK pathway activation in ST88-14 cells.

Suppl. Figure 1 from Endoglin, a Novel Biomarker and Therapeutical Target to Prevent Malignant Peripheral Nerve Sheath Tumor Growth and Metastasis

Teresa González-Muñoz, Angela Di Giannatale, Susana Garcı́a-Silva, Vanesa Santos et autres

Analysis of ENG expression and plasma circulating sEVs in human PNSTs.

Suppl. Figure 2 from Endoglin, a Novel Biomarker and Therapeutical Target to Prevent Malignant Peripheral Nerve Sheath Tumor Growth and Metastasis

Teresa González-Muñoz, Angela Di Giannatale, Susana Garcı́a-Silva, Vanesa Santos et autres

ENG knockdown leads to downregulation of pro-angiogenic and pro-metastatic gene signatures in MPNST cells.

Supplementary Data 3 from Endoglin, a Novel Biomarker and Therapeutical Target to Prevent Malignant Peripheral Nerve Sheath Tumor Growth and Metastasis

Teresa González-Muñoz, Angela Di Giannatale, Susana Garcı́a-Silva, Vanesa Santos et autres

Dual inhibition of ENG and MEK efficiently blocks ENG-Smad1/5 and MAPK/ERK pathway activation in ST88-14 cells.

Suppl. Figure 5 from Endoglin, a Novel Biomarker and Therapeutical Target to Prevent Malignant Peripheral Nerve Sheath Tumor Growth and Metastasis

Teresa González-Muñoz, Angela Di Giannatale, Susana Garcı́a-Silva, Vanesa Santos et autres

Combined anti-ENG/anti-MEK therapy inhibits MPNST growth without toxicity in vivo.

Supplementary Data 2 from Endoglin, a Novel Biomarker and Therapeutical Target to Prevent Malignant Peripheral Nerve Sheath Tumor Growth and Metastasis

Teresa González-Muñoz, Angela Di Giannatale, Susana Garcı́a-Silva, Vanesa Santos et autres

Significant deregulated genes in shENG versus shScramble STS26T cells.

Supplementary Data 1 from Endoglin, a Novel Biomarker and Therapeutical Target to Prevent Malignant Peripheral Nerve Sheath Tumor Growth and Metastasis

Teresa González-Muñoz, Angela Di Giannatale, Susana Garcı́a-Silva, Vanesa Santos et autres

Taqman probes used for quantitative PCR.

Suppl. Figure 3 from Endoglin, a Novel Biomarker and Therapeutical Target to Prevent Malignant Peripheral Nerve Sheath Tumor Growth and Metastasis

Teresa González-Muñoz, Angela Di Giannatale, Susana Garcı́a-Silva, Vanesa Santos et autres

ENG knockdown does not affect MPNST cell apoptosis in vivo.

Suppl. Figure 4 from Endoglin, a Novel Biomarker and Therapeutical Target to Prevent Malignant Peripheral Nerve Sheath Tumor Growth and Metastasis

Teresa González-Muñoz, Angela Di Giannatale, Susana Garcı́a-Silva, Vanesa Santos et autres

Anti-ENG therapy is active against MPNST xenografts.