Sphingosine induces apoptosis and down-regulation of MYCN in PAX3-FOXO1-positive alveolar rhabdomyosarcoma cells irrespective of TP53 mutation

Eun Hyun Ahn, Michael B. Lee, Dong Joo Seo, Juseong Lee, Yonghyun Kim, Kshitiz Gupta

Research output: Contribution to journalArticlepeer-review

Abstract

Background/Aim: Rhabdomyosarcoma is the most common type of pediatric soft-tissue sarcoma. Among the subsets of this disease, alveolar rhabdomyosarcoma (ARMS) expressing paired box 3 (PAX3) and forkhead box O1 (PAX3-FOXO1) fusion oncoprotein has the worst prognosis. The goal of this study was to investigate the chemotherapeutic effects of sphingosine on PAX3-FOXO1-positive ARMS cells [tumor protein p53 (TP53)-mutated RH30 and TP53 wild-type RH18 cells]. Materials and Methods: The proliferation, cell death, apoptosis, cell cycle, and MYCN proto-oncogene (MYCN) expression of RH30 and RH18 cells were determined. Results: Sphingosine inhibited the growth and caused cell death in a dose-dependent manner in both cell lines. Sphingosine triggered cell death by inducing apoptosis without affecting the cell cycle. MYCN expression was down-regulated within 2 and 4 h of sphingosine treatment in both RH30 and RH18 cells. Conclusion: Sphingosine exerts antiproliferative and pro-apoptotic effects via MYCN down-regulation independently of TP53 mutation status in PAX3-FOXO1-positive ARMS cells.

Original languageEnglish (US)
Pages (from-to)71-76
Number of pages6
JournalAnticancer research
Volume38
Issue number1
DOIs
StatePublished - Jan 2018
Externally publishedYes

Keywords

  • Alveolar rhabdomyosarcoma (ARMS)
  • Apoptosis
  • Gene expression
  • MYCN
  • PAX3-FOXO1
  • Sphingosine
  • TP53

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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