<i>Neurofibromatosis Type 1</i> Gene Alterations Define Specific Features of a Subset of Glioblastomas
<i>Neurofibromatosis type 1</i> (<i>NF1</i>) gene mutations or alterations occur within neurofibromatosis type 1 as well as in many different malignant tumours on the somatic level. In glioblastoma, <i>NF1</i> loss of function plays a major role in inducing the me...
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2021-12-01
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| Series: | International Journal of Molecular Sciences |
| Subjects: | |
| Online Access: | https://www.mdpi.com/1422-0067/23/1/352 |
| Summary: | <i>Neurofibromatosis type 1</i> (<i>NF1</i>) gene mutations or alterations occur within neurofibromatosis type 1 as well as in many different malignant tumours on the somatic level. In glioblastoma, <i>NF1</i> loss of function plays a major role in inducing the mesenchymal (MES) subtype and, therefore defining the most aggressive glioblastoma. This is associated with an immune signature and mediated via the NF1–MAPK–FOSL1 axis. Specifically, increased invasion seems to be regulated via mutations in the leucine-rich domain (LRD) of the <i>NF1</i> gene product neurofibromin. Novel targets for therapy may arise from neurofibromin deficiency-associated cellular mechanisms that are summarised in this review. |
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| ISSN: | 1661-6596 1422-0067 |