Data Descriptor: Pharmacological and genomic profiling of neurofibromatosis type 1 plexiform neurofibroma-derived schwann cells

Marc Ferrer, Sara J.C. Gosline, Marigo Stathis, Xiaohu Zhang, Xindi Guo, Rajarshi Guha, Dannielle A. Ryman, Margaret R. Wallace, Laura Kasch-Semenza, Haiping Hao, Roxann Ingersoll, David Mohr, Craig Thomas, Sharad Verma, Justin Guinney, Jaishri Blakeley

Research output: Contribution to journalArticle

Abstract

Neurofibromatosis type I (NF1) is an autosomal dominant genetic condition characterized by peripheral nervous system tumors (PNSTs), including plexiform neurofibromas (pNFs) that cause nerve dysfunction, deformity, pain damage to adjacent structures, and can undergo malignant transformation. There are no effective therapies to prevent or treat pNFs. Drug discovery efforts are slowed by the 'benign' nature of the Schwann cells that are the progenitor cells of pNF. In this work we characterize a set of pNF-derived cell lines at the genomic level (via SNP Arrays, RNAseq, and Whole Exome- Sequencing), and carry out dose response-based quantitative high-throughput screening (qHTS) with a collection of 1,912 oncology-focused compounds in a 1536-well microplate cell proliferation assays. Through the characterization and screening of NF1(-/-), NF1(+/+) and NF1(+/-) Schwann cell lines, this resource introduces novel therapeutic avenues for the development for NF1 associated pNF as well as all solid tumors with NF1 somatic mutations. The integrated data sets are openly available for further analysis at http://www.synapse.org/pnf Cell Culture.

Original languageEnglish (US)
Article number180106
JournalScientific data
Volume5
DOIs
StatePublished - Jun 12 2018

ASJC Scopus subject areas

  • Statistics and Probability
  • Information Systems
  • Education
  • Computer Science Applications
  • Statistics, Probability and Uncertainty
  • Library and Information Sciences

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    Ferrer, M., Gosline, S. J. C., Stathis, M., Zhang, X., Guo, X., Guha, R., Ryman, D. A., Wallace, M. R., Kasch-Semenza, L., Hao, H., Ingersoll, R., Mohr, D., Thomas, C., Verma, S., Guinney, J., & Blakeley, J. (2018). Data Descriptor: Pharmacological and genomic profiling of neurofibromatosis type 1 plexiform neurofibroma-derived schwann cells. Scientific data, 5, [180106]. https://doi.org/10.1038/sdata.2018.106