Neurofibromatosis: Molecular Pathogenesis and Natural Compounds as Potential Treatments
The neurofibromatosis syndromes, including NF1, NF2, and schwannomatosis, are tumor suppressor syndromes characterized by multiple nervous system tumors, particularly Schwann cell neoplasms. NF-related tumors are mainly treated by surgery, and some of them have been treated by but are refractory to...
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doaj-3619a9d037e049dcbaa7938cfc6cce892021-09-17T05:53:37ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2021-09-011110.3389/fonc.2021.698192698192Neurofibromatosis: Molecular Pathogenesis and Natural Compounds as Potential TreatmentsAnusha Amaravathi0Anusha Amaravathi1Janet L. Oblinger2D. Bradley Welling3A. Douglas Kinghorn4Long-Sheng Chang5Long-Sheng Chang6Long-Sheng Chang7Center for Childhood Cancer and Blood Diseases, Abigail Wexner Research Institute at Nationwide Children’s Hospital, Columbus, OH, United StatesDepartment of Medicine, University of Rochester School of Medicine and Dentistry, Rochester, NY, United StatesCenter for Childhood Cancer and Blood Diseases, Abigail Wexner Research Institute at Nationwide Children’s Hospital, Columbus, OH, United StatesDepartment of Otolaryngology Head & Neck Surgery, Harvard Medical School, Massachusetts Eye and Ear, and Massachusetts General Hospital, Boston, MA, United StatesDivision of Medicinal Chemistry and Pharmacognosy, The Ohio State University College of Pharmacy, Columbus, OH, United StatesCenter for Childhood Cancer and Blood Diseases, Abigail Wexner Research Institute at Nationwide Children’s Hospital, Columbus, OH, United StatesDepartment of Pediatrics, The Ohio State University College of Medicine, Columbus, OH, United StatesDepartment of Otolaryngology-Head & Neck Surgery, The Ohio State University College of Medicine, Columbus, OH, United StatesThe neurofibromatosis syndromes, including NF1, NF2, and schwannomatosis, are tumor suppressor syndromes characterized by multiple nervous system tumors, particularly Schwann cell neoplasms. NF-related tumors are mainly treated by surgery, and some of them have been treated by but are refractory to conventional chemotherapy. Recent advances in molecular genetics and genomics alongside the development of multiple animal models have provided a better understanding of NF tumor biology and facilitated target identification and therapeutic evaluation. Many targeted therapies have been evaluated in preclinical models and patients with limited success. One major advance is the FDA approval of the MEK inhibitor selumetinib for the treatment of NF1-associated plexiform neurofibroma. Due to their anti-neoplastic, antioxidant, and anti-inflammatory properties, selected natural compounds could be useful as a primary therapy or as an adjuvant therapy prior to or following surgery and/or radiation for patients with tumor predisposition syndromes, as patients often take them as dietary supplements and for health enhancement purposes. Here we review the natural compounds that have been evaluated in NF models. Some have demonstrated potent anti-tumor effects and may become viable treatments in the future.https://www.frontiersin.org/articles/10.3389/fonc.2021.698192/fullneurofibromatosis (NF)signaling pathwaytargeted therapynatural compoundseIF4A inhibitorsrocaglamide |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Anusha Amaravathi Anusha Amaravathi Janet L. Oblinger D. Bradley Welling A. Douglas Kinghorn Long-Sheng Chang Long-Sheng Chang Long-Sheng Chang |
spellingShingle |
Anusha Amaravathi Anusha Amaravathi Janet L. Oblinger D. Bradley Welling A. Douglas Kinghorn Long-Sheng Chang Long-Sheng Chang Long-Sheng Chang Neurofibromatosis: Molecular Pathogenesis and Natural Compounds as Potential Treatments Frontiers in Oncology neurofibromatosis (NF) signaling pathway targeted therapy natural compounds eIF4A inhibitors rocaglamide |
author_facet |
Anusha Amaravathi Anusha Amaravathi Janet L. Oblinger D. Bradley Welling A. Douglas Kinghorn Long-Sheng Chang Long-Sheng Chang Long-Sheng Chang |
author_sort |
Anusha Amaravathi |
title |
Neurofibromatosis: Molecular Pathogenesis and Natural Compounds as Potential Treatments |
title_short |
Neurofibromatosis: Molecular Pathogenesis and Natural Compounds as Potential Treatments |
title_full |
Neurofibromatosis: Molecular Pathogenesis and Natural Compounds as Potential Treatments |
title_fullStr |
Neurofibromatosis: Molecular Pathogenesis and Natural Compounds as Potential Treatments |
title_full_unstemmed |
Neurofibromatosis: Molecular Pathogenesis and Natural Compounds as Potential Treatments |
title_sort |
neurofibromatosis: molecular pathogenesis and natural compounds as potential treatments |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Oncology |
issn |
2234-943X |
publishDate |
2021-09-01 |
description |
The neurofibromatosis syndromes, including NF1, NF2, and schwannomatosis, are tumor suppressor syndromes characterized by multiple nervous system tumors, particularly Schwann cell neoplasms. NF-related tumors are mainly treated by surgery, and some of them have been treated by but are refractory to conventional chemotherapy. Recent advances in molecular genetics and genomics alongside the development of multiple animal models have provided a better understanding of NF tumor biology and facilitated target identification and therapeutic evaluation. Many targeted therapies have been evaluated in preclinical models and patients with limited success. One major advance is the FDA approval of the MEK inhibitor selumetinib for the treatment of NF1-associated plexiform neurofibroma. Due to their anti-neoplastic, antioxidant, and anti-inflammatory properties, selected natural compounds could be useful as a primary therapy or as an adjuvant therapy prior to or following surgery and/or radiation for patients with tumor predisposition syndromes, as patients often take them as dietary supplements and for health enhancement purposes. Here we review the natural compounds that have been evaluated in NF models. Some have demonstrated potent anti-tumor effects and may become viable treatments in the future. |
topic |
neurofibromatosis (NF) signaling pathway targeted therapy natural compounds eIF4A inhibitors rocaglamide |
url |
https://www.frontiersin.org/articles/10.3389/fonc.2021.698192/full |
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