New Model Systems and the Development of Targeted Therapies for the Treatment of Neurofibromatosis Type 1-Associated Malignant Peripheral Nerve Sheath Tumors

Neurofibromatosis Type 1 (NF1) is a common genetic disorder and cancer predisposition syndrome (1:3000 births) caused by mutations in the tumor suppressor gene . encodes neurofibromin, a negative regulator of the Ras signaling pathway. Individuals with NF1 often develop benign tumors of the peripher...

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Veröffentlicht in:	Genes 2020-04, Vol.11 (5), p.477
Hauptverfasser:	Williams, Kyle B, Largaespada, David A
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Benign Cell Lineage - genetics Chemotherapy Clinical trials CRISPR Cytotoxicity Disease Models, Animal Drug screening Genes Genetic disorders Genetic screening Growth factors Heterografts Humans Lethality Ligands MAP kinase MEK inhibitors Mice Molecular Targeted Therapy Mutagenesis Mutation Mutation - genetics Nervous system Neurofibromatosis Neurofibromatosis 1 - complications Neurofibromatosis 1 - genetics Neurofibromatosis 1 - pathology Neurofibromin 1 Neurofibromin 1 - genetics Neurofibrosarcoma - complications Neurofibrosarcoma - genetics Neurofibrosarcoma - pathology Neurological disorders Patients Peripheral nerves Protein kinase Proteins Quality of life Ras protein Recklinghausen's disease Review Schwann cells Schwann Cells - pathology Senescence Signal transduction Therapeutic applications Tumor suppressor genes Tumors
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