The role of TP53 gain-of-function mutation in multifocal glioblastoma

J Neurooncol. 2020 Mar;147(1):37-47. doi: 10.1007/s11060-019-03318-5. Epub 2020 Jan 30.

Abstract

Purpose: The phenotypic and genotypic landscapes in multifocal glioblastoma (MF GBM) cases can vary greatly among lesions. In a MF GBM patient, the rapid development of a secondary lesion was investigated to determine if a unique genetic signature could account for the apparent increased malignancy of this lesion.

Methods: The primary (G52) and secondary (G53) tumours were resected to develop patient derived models followed by functional assays and multiplatform molecular profiling.

Results: Molecular profiling revealed G52 was wild-type for TP53 while G53 presented with a TP53 missense mutation. Functional studies demonstrated increased proliferation, migration, invasion and colony formation in G53.

Conclusion: This data suggests that the TP53 mutation led to gain-of-function phenotypes and resulted in greater overall oncogenic potential of G53.

Keywords: Gain-of-function; Glioblastoma; Multifocal; TP53.

MeSH terms

  • Biomarkers, Tumor / genetics
  • Brain Neoplasms / genetics*
  • Brain Neoplasms / pathology
  • Gain of Function Mutation
  • Gene Expression Regulation, Neoplastic*
  • Glioblastoma / genetics*
  • Glioblastoma / pathology
  • Humans
  • Ki-67 Antigen / metabolism
  • Male
  • Middle Aged
  • Mutation
  • Tumor Cells, Cultured
  • Tumor Suppressor Protein p53 / genetics*

Substances

  • Biomarkers, Tumor
  • Ki-67 Antigen
  • TP53 protein, human
  • Tumor Suppressor Protein p53