Suppression of RIZ in biologically unfavourable neuroblastomas

Janos Geli, Nimrod Kiss, Per Kogner, Catharina Larsson

Research output: Contribution to journalArticlepeer-review


Neuroblastoma is a paediatric solid tumor characterized by recurrent genomic abnormalities of prognostic importance. One of the most commonly observed abnormalities is deletion of the short arm of chromosome 1 and reduced expression of cancer related genes in this chromosomal arm. The long isoform of the retinoblastoma protein-interacting zink finger gene (RIZ1) is a known tumor suppressor and a candidate neuroblastoma gene located at 1p36.2. The present study was undertaken to further assess the possible involvement of RIZ in neuroblastoma development. Expression of RIZ transcripts were quantified in a panel of neuroblastoma cell lines and tumors (33 neuroblastomas and 3 ganglioneuromas). Methylation status of promoter P1 driving RIZ1 expression was quantified by bisulfite Pyrosequencing. Only low mean levels of promoter methylation (<10%) were observed in all samples. However, RIZ1 and RIZ1+2 mRNA were significantly under-expressed in biologically unfavourable tumors characterized by 1p loss (p<0.005) or MYCN amplification (p<0.005). Suppression of RIZ1 is likely to contribute to the pathogenesis of biologically unfavourable neuroblastomas. In contrast to multiple other neoplasias, RIZ1 promoter methylation is not a common event in neuroblastoma.

Original languageEnglish (US)
Pages (from-to)1323-1330
Number of pages8
JournalInternational journal of oncology
Issue number5
StatePublished - Nov 2010
Externally publishedYes


  • MRNA expression
  • Neuroblastoma
  • Promoter methylation
  • RIZ

ASJC Scopus subject areas

  • Oncology
  • Cancer Research


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