Neurofibromatosis Type 1 Gene Alterations Define Specific Features of a Subset of Glioblastomas

( ) gene mutations or alterations occur within neurofibromatosis type 1 as well as in many different malignant tumours on the somatic level. In glioblastoma, loss of function plays a major role in inducing the mesenchymal (MES) subtype and, therefore defining the most aggressive glioblastoma. This i...

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Bibliographic Details
Published in:	International journal of molecular sciences 2021-12, Vol.23 (1), p.352
Main Authors:	Scheer, Maximilian, Leisz, Sandra, Sorge, Eberhard, Storozhuk, Olha, Prell, Julian, Ho, Ivy, Harder, Anja
Format:	Article
Language:	eng
Subjects:	Animals Brain cancer Chromosomes DNA methylation Genes, Neurofibromatosis 1 Genetic disorders Glioblastoma Glioblastoma - genetics Humans invasiveness Kinases MAP kinase mesenchymal Mesenchyme Mesoderm - pathology Models, Biological Mutation Mutation - genetics Neurofibromatosis Neurofibromatosis 1 - genetics neurofibromin Neurofibromin 1 Neurological disorders NF1 Review Tumors
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Summary:	( ) gene mutations or alterations occur within neurofibromatosis type 1 as well as in many different malignant tumours on the somatic level. In glioblastoma, 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 gene product neurofibromin. Novel targets for therapy may arise from neurofibromin deficiency-associated cellular mechanisms that are summarised in this review.
ISSN:	1422-0067 1661-6596 1422-0067