Logo image
Sonic Hedgehog promotes proliferation of Notch-dependent monociliated choroid plexus tumour cells
Journal article   Peer reviewed

Sonic Hedgehog promotes proliferation of Notch-dependent monociliated choroid plexus tumour cells

Li Li, Katie B Grausam, Jun Wang, Melody P Lun, Jasmin Ohli, Hart G W Lidov, Monica L Calicchio, Erliang Zeng, Jeffrey L Salisbury, Robert J Wechsler-Reya, …
Nature cell biology, Vol.18(4), pp.418-430
04/2016
DOI: 10.1038/ncb3327
PMCID: PMC4814324
PMID: 26999738

View Online

Abstract

Aberrant Notch signalling has been linked to many cancers including choroid plexus (CP) tumours, a group of rare and predominantly paediatric brain neoplasms. We developed animal models of CP tumours, by inducing sustained expression of Notch1, that recapitulate properties of human CP tumours with aberrant NOTCH signalling. Whole-transcriptome and functional analyses showed that tumour cell proliferation is associated with Sonic Hedgehog (Shh) in the tumour microenvironment. Unlike CP epithelial cells, which have multiple primary cilia, tumour cells possess a solitary primary cilium as a result of Notch-mediated suppression of multiciliate differentiation. A Shh-driven signalling cascade in the primary cilium occurs in tumour cells but not in epithelial cells. Lineage studies show that CP tumours arise from monociliated progenitors in the roof plate characterized by elevated Notch signalling. Abnormal SHH signalling and distinct ciliogenesis are detected in human CP tumours, suggesting the SHH pathway and cilia differentiation as potential therapeutic avenues.
Oligonucleotide Array Sequence Analysis Humans Choroid Plexus - metabolism Gene Expression Regulation, Neoplastic Hedgehog Proteins - metabolism Male Gene Expression Profiling Choroid Plexus - ultrastructure Mice, 129 Strain Tumor Microenvironment - genetics Hedgehog Proteins - genetics Choroid Plexus Neoplasms - genetics Cilia - ultrastructure Female Tumor Cells, Cultured Microscopy, Electron, Transmission Cell Proliferation - genetics Choroid Plexus - pathology Mice, Inbred C57BL Mice, Transgenic Signal Transduction - genetics Choroid Plexus Neoplasms - pathology Receptor, Notch1 - metabolism Reverse Transcriptase Polymerase Chain Reaction Blotting, Western Cilia - metabolism Microscopy, Confocal Animals Choroid Plexus Neoplasms - metabolism Receptor, Notch1 - genetics

Details

Logo image