Molecular Mechanisms and Clinical Treatments of Neurofibromatosis Type 1 Related Tumors
A special issue of Cells (ISSN 2073-4409). This special issue belongs to the section "Cells of the Nervous System".
Deadline for manuscript submissions: 31 August 2024 | Viewed by 1000
Special Issue Editors
Interests: mitochondrial pathophysiology; tumor metabolism; molecular chaperones
Special Issue Information
Dear Colleagues,
Neurofibromatosis type 1 (NF1) is a tumor-predisposing genetic syndrome where inactivating mutations in the NF1 gene elicit Ras signaling dysregulation and prime the onset of benign tumors in central and peripheral nerves. Ras hyperactivation drives tumor growth by orchestrating changes in biochemical and metabolic circuits of transformed cells with an altered network of interactions in the tumor microenvironment. The dysregulated crosstalk between tumor cells and environmental cues prompts further (epi)genetic lesions and the transition towards malignancy, as exemplified by the evolution from neurofibromas, i.e., benign Schwann cell tumors, to malignant peripheral nerve sheath tumors. Hence, NF1 constitutes an extraordinary model that is used to study progression from early tumor stages to aggressive neoplastic forms. In spite of the huge advances made in the comprehension of this process, translation to effective therapies remains limited, and the clinical settings of NF1 patients warrant major improvements.
This Special Issue aims to draw a comprehensive picture of the biological determinants that support the pro-tumor activity of Ras signaling along the stepwise transformation towards malignancy. The integration of this information into the clinical management of NF1 patients can trailblaze more advanced and effective therapeutic options, and can establish the basis for studying the complex interactions that induce neoplastic progression in a broader set of Ras-related tumor models.
Dr. Andrea Rasola
Dr. Federica Chiara
Guest Editors
Manuscript Submission Information
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Keywords
- neurofibromatosis type 1
- rare tumors
- genetic syndrome
- plexiform neurofibroma
- malignant peripheral nerve sheath tumor
- tumor suppressor
- Ras signalling
- tumor microenvironment
- anti-neoplastic therapy
- neoplastic transformation
