Table 1.

Genomic characteristics and SP-2509 sensitivity of the ES cell line cohort

Cell lineHistologyFusion geneTP53 statusSTAG2 statusa,bKDM1A statusaKDM1A mRNA expression72-hr SP-2509 IC50 (μmol/L)144-hr GSK-LSD1 IC50 (μmol/L)
SK-N-MCESEWS/FLI Type ITruncationWild-typeUN2.80 ± 0.040.081 ± 0.002ND
A673ESEWS/FLI Type IQ119fsWild-typeWild-type8.49 ± 1.290.109 ± 0.030>330
TC252ESEWS/FLI Type IWild-typeUNWild-type2.23 ± 0.070.183 ± 0.017266.23 ± 12.99
TC32ESEWS/FLI Type IWild-typeI636fsWild-type2.32 ± 0.230.226 ± 0.032221.67 ± 18.03
SK-ES-1ESEWS/FLI Type IIC176FQ735*UN7.08 ± 1.470.418 ± 0.017ND
ES-4ESEWS/FLI Type IIWild-typeWild-typeUN2.15 ± 0.300.502 ± 0.101>330
TTC-466ESEWS/ERGR175GNull expWild-type7.10 ± 2.270.536 ± 0.078>330
CHLA-9ESEWS/FLI Type IWild-typeV628insTDIUN2.75 ± 0.270.547 ± 0.029ND
VH64ESEWS/FLI Type IIWild-typeUNUN1.18 ± 0.190.561 ± 0.043ND
EW-8ESEWS/FLI Type IY220CN475fsWild-type3.52 ± 0.200.568 ± 0.031ND
CADO-ES1ESEWS/ERGWild-typeWild-typeWild-type3.92 ± 1.210.593 ± 0.068ND
CHLA-258ESEWS/FLI Type IIIWild-typeWild-typeWild-type0.67 ± 0.020.810 ± 0.126300.14 ± 43.82
RD-ESESEWS/FLI Type IIR273CNull expWild-type5.66 ± 0.750.857 ± 0.068ND
EWS-502ESEWS/FLI Type IC135FNull expWild-type4.99 ± 0.170.902 ± 0.078>330
CHLA-10ESEWS/FLI Type INFUNUN2.94 ± 0.260.952 ± 0.060>330
ES-2ESEWS/FLI Type IIIR175HE523*UN1.98 ± 0.221.128 ± 0.126>330
TC-71ESEWS/FLI Type IR213*Wild-typeWild-type3.14 ± 0.501.593 ± 0.036ND
hMSChMSCUNUNUN>4ND
TIP5HUFFUNUNUN18.20 ± 2.131ND
  • Abbreviations: ES, Ewing sarcoma; hMSC, Primary human mesenchymal stem cells; HUFF, Human foreskin fibroblasts; ND, Not determined; NF, Non- functional; UN, Unknown; Null exp, No expression.

  • Fusion gene: Type I (Exon 7 EWS/Exon 6 of FLI1), Type II (Exon 7 EWS/Exon 5 FLI1), and Type III (Exon 10 EWS/Exon 6 FLI-1).

  • aCrompton et al. (6).

  • bBrohl et al. (5).