Chapter title |
Malignant Peripheral Nerve Sheath Tumors
|
---|---|
Chapter number | 22 |
Book title |
Cancer and Zebrafish
|
Published in |
Advances in experimental medicine and biology, May 2016
|
DOI | 10.1007/978-3-319-30654-4_22 |
Pubmed ID | |
Book ISBNs |
978-3-31-930652-0, 978-3-31-930654-4
|
Authors |
Adam D. Durbin, Dong Hyuk Ki, Shuning He, A. Thomas Look |
Editors |
David M. Langenau |
Abstract |
Malignant peripheral nerve sheath tumors (MPNST) are tumors derived from Schwann cells or Schwann cell precursors. Although rare overall, the incidence of MPNST has increased with improved clinical management of patients with the neurofibromatosis type 1 (NF1) tumor predisposition syndrome. Unfortunately, current treatment modalities for MPNST are limited, with no targeted therapies available and poor efficacy of conventional radiation and chemotherapeutic regimens. Many murine and zebrafish models of MPNST have been developed, which have helped to elucidate the genes and pathways that are dysregulated in MPNST tumorigenesis, including the p53, and the RB1, PI3K-Akt-mTOR, RAS-ERK and Wnt signaling pathways. Preclinical results have suggested that new therapies, including mTOR and ERK inhibitors, may synergize with conventional chemotherapy in human tumors. The discovery of new genome editing technologies, like CRISPR-cas9, and their successful application to the zebrafish model will enable rapid progress in the faithful modeling of MPNST molecular pathogenesis. The zebrafish model is especially suited for high throughput screening of new targeted therapeutics as well as drugs approved for other purposes, which may help to bring enhanced treatment modalities into human clinical trials for this devastating disease. |
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Geographical breakdown
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Demographic breakdown
Readers by professional status | Count | As % |
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Student > Master | 7 | 23% |
Researcher | 4 | 13% |
Other | 3 | 10% |
Professor > Associate Professor | 3 | 10% |
Student > Doctoral Student | 2 | 7% |
Other | 7 | 23% |
Unknown | 4 | 13% |
Readers by discipline | Count | As % |
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Neuroscience | 4 | 13% |
Veterinary Science and Veterinary Medicine | 2 | 7% |
Other | 3 | 10% |
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