URL: | http://www.synapse.org/pnfCellCulture |
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Description: | Welcome to the data portal that contains the high throughput data generated from plexiform neurofibroma cell lines. This portal was designed integrate data across a joint effort between Johns Hopkins and NCATS to profile and screen a set of cell lines to identify potential drugs that could abate plexiform growth in NF1 Patients. |
Year founded: | 2018 |
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Country/Region: | United States |
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University/Institution: | Johns Hopkins University |
Address: | Neurofibromatosis Therapeutic Acceleration Program (NTAP), Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA |
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Country/Region: | United States |
Contact name (PI/Team): | Jaishri O. Blakeley |
Contact email (PI/Helpdesk): | jblakakel3@jhmi.edu |
Pharmacological and genomic profiling of neurofibromatosis type 1 plexiform neurofibroma-derived schwann cells. [PMID: 29893754]
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/pnfCellCulture. |