Book section
Molecular Genetic Studies of Pancreatic Neuroendocrine Tumors: New Therapeutic Approaches
- Abstract:
- Pancreatic neuroendocrine tumors (PNETs) can occur as sporadic neoplasms or as part of hereditary syndromes such as multiple endocrine neoplasia type 1 (MEN1). MEN1, which is an autosomal dominant disorder, is due to loss-of-function mutations of the tumor suppressor MEN1 gene that encodes menin. Approximately 40% of nonfamilial (ie, sporadic) PNETs have MEN1 mutations, with subsequent loss of menin, which acts as a tumor suppressor. Menin is a scaffold protein with roles in transcriptional regulation, genome stability, DNA repair, protein degradation, cell motility and adhesion, microRNA biogenesis, cell division, cell cycle control, epigenetic regulation, and Wnt signaling. Emerging therapies targeting the functional roles of menin with Men1 gene replacement therapy, epigenetic modulators, and antagonists of Wnt-signaling may prove useful for future treatment of PNETs.
- Publication status:
- Published
- Peer review status:
- Peer reviewed
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- Files:
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(Preview, Accepted manuscript, pdf, 551.1KB, Terms of use)
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- Publisher copy:
- 10.1016/j.ecl.2018.04.007
Authors
- Publisher:
- Elsevier
- Host title:
- Endocrinology and Metabolism Clinics of North America
- Publication date:
- 2018-08-08
- DOI:
- ISSN:
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0889-8529
- Keywords:
- Pubs id:
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pubs:857229
- UUID:
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uuid:60df3b77-b1b7-4623-80a0-57ba33ce58d9
- Local pid:
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pubs:857229
- Source identifiers:
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857229
- Deposit date:
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2018-06-15
- ARK identifier:
Terms of use
- Copyright holder:
- Elsevier
- Copyright date:
- 2018
- Notes:
- © 2018 Elsevier Inc. All rights reserved. This is the Accepted Manuscript version of the article. The final version is available online from Elsevier at: https://doi.org/10.1016/j.ecl.2018.04.007
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