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Notch3 signaling initiates choroid plexus tumor formation

L Dang1, X Fan, A Chaudhry

  • 1Institute for Cell Engineering, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.

Oncogene
|September 28, 2005
PubMed

Insights

Activated Notch3 acts as an oncogene, causing choroid plexus tumors (CPTs) in mice. This research links the Notch pathway to human CPT development, highlighting its role in brain tumor formation.

Area of Science:

  • Neuro-oncology
  • Developmental Biology
  • Molecular Biology

Background:

  • The role of Notch3 in brain tumor formation is not well understood.
  • Previous studies focused on Notch3's role in brain development.

Purpose of the Study:

  • To investigate the potential oncogenic role of Notch3 in brain tumorigenesis.
  • To determine if the Notch pathway is involved in human choroid plexus tumor (CPT) pathogenesis.

Main Methods:

  • Constitutively active Notch3 was introduced into periventricular cells of embryonic mice.
  • Tumor formation, characteristics, and proliferation rates were analyzed.
  • Notch pathway activity and receptor mRNA levels were assessed in mouse and human CPTs.

Main Results:

  • Constitutively active Notch3 induced CPTs in 83% of mice, often with hydrocephalus.
  • Mouse CPTs were histologically similar to human choroid plexus papillomas, with 4-6% proliferation.
  • Elevated Notch pathway activity and receptor mRNA levels were observed in human CPTs.

Conclusions:

  • Activated Notch3 can function as an oncogene in the developing brain.
  • The Notch pathway is implicated in the pathogenesis of human choroid plexus tumors.
  • Notch2 overexpression suggests pathway involvement beyond Notch3 in CPTs.

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