An integrated approach identifies Nhlh1 and Insm1 as sonic hedgehog-regulated genes in developing cerebellum and medulloblastoma

Enrico De Smaele, Caterina Fragomeli, Elisabetta Ferretti, Marianna Pelloni, Agnese Po, Gianluca Canettieri, Sonia Coni, Lucia Di Marcotullio, Azzura Greco, Marta Moretti, Concezio Di Rocco, Simona Pazzaglia, Marella Maroder, Isabella Screpanti, Giuseppe Giannini, Alberto Gulino

Research output: Contribution to journalArticle

Abstract

Medulloblastoma (MB) is the most common malignant brain tumor of childhood arising from deregulated cerebellar development. Sonic Hedgehog (Shh) pathway plays a critical role in cerebellar development and its aberrant expression has been identified in MB. Gene expression profiling of cerebella from 1- to 14-day-old mice unveiled a cluster of genes whose expression correlates with the levels of Hedgehog (HH) activity. From this cluster, we identified Insm1 and Nhlh1/NSCL1 as novel HH targets induced by Shh treatment in cultured cerebellar granule cell progenitors. Nhlh1 promoter was found to be bound and activated by Gli1 transcription factor. Remarkably, the expression of these genes is also upregulated in mouse and human HH-dependent MBs, suggesting that they may be either a part of the HH-induced tumorigenic process or a specific trait of HH-dependent tumor cells.

Original languageEnglish
Pages (from-to)89-98
Number of pages10
JournalNeoplasia (United States)
Volume10
Issue number1
DOIs
Publication statusPublished - Jan 2008

ASJC Scopus subject areas

  • Cancer Research

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    De Smaele, E., Fragomeli, C., Ferretti, E., Pelloni, M., Po, A., Canettieri, G., Coni, S., Di Marcotullio, L., Greco, A., Moretti, M., Di Rocco, C., Pazzaglia, S., Maroder, M., Screpanti, I., Giannini, G., & Gulino, A. (2008). An integrated approach identifies Nhlh1 and Insm1 as sonic hedgehog-regulated genes in developing cerebellum and medulloblastoma. Neoplasia (United States), 10(1), 89-98. https://doi.org/10.1593/neo.07891