TY - JOUR T1 - Sphingosine Induces Apoptosis and Down-regulation of <em>MYCN</em> in PAX3–FOXO1-positive Alveolar Rhabdomyosarcoma Cells Irrespective of <em>TP53</em> Mutation JF - Anticancer Research JO - Anticancer Res SP - 71 LP - 76 VL - 38 IS - 1 AU - EUN HYUN AHN AU - MICHAEL B. LEE AU - DONG JOO SEO AU - JUSEONG LEE AU - YONGHYUN KIM AU - KSHITIZ GUPTA Y1 - 2018/01/01 UR - http://ar.iiarjournals.org/content/38/1/71.abstract N2 - 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. ER -