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WT1 Alternative Splicing: Role of Its Isoforms in Neuroblastoma

Daniela Maria Rasà1, Agata Grazia D'Amico1,2, Grazia Maugeri1

  • 1Sections of Human Anatomy and Histology, Department of Biomedical and Biotechnological Sciences, University of Catania, Via S. Sofia, 87, 95123, Catania, Italy.

Journal of Molecular Neuroscience : MN
|May 24, 2017
PubMed

Insights

The Wilms tumor 1 (WT1) gene acts as both a tumor suppressor and oncogene. Further research into its variants could reveal new cancer markers and therapies, particularly for neuroblastoma.

Area of Science:

  • Oncology
  • Molecular Biology
  • Genetics

Background:

  • Wilms tumor 1 (WT1) is a tumor suppressor gene implicated in various cancers beyond its namesake renal neoplasm.
  • WT1 exhibits dual roles, functioning as both a pro-oncogene and an anti-oncogene, leading to unclear functional significance.
  • Over 14 WT1 transcriptional variants exist, but research has predominantly focused on a limited subset of isoforms.

Purpose of the Study:

  • To describe the structural features of WT1 variants.
  • To review existing evidence on the involvement of WT1 isoforms in cancer, with a specific focus on neuroblastoma.
  • To highlight the potential for future research in fully characterizing WT1 isoforms for clinical applications.

Main Methods:

  • Literature review of WT1 structural features and cancer involvement.
  • Analysis of existing evidence linking WT1 variants to oncogenesis.
  • Emphasis on neuroblastoma as a model for WT1 isoform research.

Main Results:

  • Detailed structural characteristics of various WT1 transcriptional variants are presented.
  • Evidence is reviewed demonstrating the differential roles of WT1 isoforms in various cancers, including neuroblastoma.
  • The complexity of WT1 function is highlighted due to its context-dependent oncogenic or tumor-suppressive activities.

Conclusions:

  • Comprehensive characterization of all WT1 isoforms is crucial for understanding their roles in tumorigenesis.
  • Future investigations into WT1 variants may uncover novel molecular tumor markers.
  • Targeting specific WT1 isoforms presents a potential therapeutic strategy for cancer treatment.
Keywords:
Alternative splicingIsoformsNeuroblastomaWT1

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