Table 1.

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

Cell line	Histology	Fusion gene	TP53 status	STAG2 status^a^,^b	KDM1A status^a	KDM1A mRNA expression	72-hr SP-2509 IC₅₀ (μmol/L)	144-hr GSK-LSD1 IC₅₀ (μmol/L)
SK-N-MC	ES	EWS/FLI Type I	Truncation	Wild-type	UN	2.80 ± 0.04	0.081 ± 0.002	ND
A673	ES	EWS/FLI Type I	Q119fs	Wild-type	Wild-type	8.49 ± 1.29	0.109 ± 0.030	>330
TC252	ES	EWS/FLI Type I	Wild-type	UN	Wild-type	2.23 ± 0.07	0.183 ± 0.017	266.23 ± 12.99
TC32	ES	EWS/FLI Type I	Wild-type	I636fs	Wild-type	2.32 ± 0.23	0.226 ± 0.032	221.67 ± 18.03
SK-ES-1	ES	EWS/FLI Type II	C176F	Q735*	UN	7.08 ± 1.47	0.418 ± 0.017	ND
ES-4	ES	EWS/FLI Type II	Wild-type	Wild-type	UN	2.15 ± 0.30	0.502 ± 0.101	>330
TTC-466	ES	EWS/ERG	R175G	Null exp	Wild-type	7.10 ± 2.27	0.536 ± 0.078	>330
CHLA-9	ES	EWS/FLI Type I	Wild-type	V628insTDI	UN	2.75 ± 0.27	0.547 ± 0.029	ND
VH64	ES	EWS/FLI Type II	Wild-type	UN	UN	1.18 ± 0.19	0.561 ± 0.043	ND
EW-8	ES	EWS/FLI Type I	Y220C	N475fs	Wild-type	3.52 ± 0.20	0.568 ± 0.031	ND
CADO-ES1	ES	EWS/ERG	Wild-type	Wild-type	Wild-type	3.92 ± 1.21	0.593 ± 0.068	ND
CHLA-258	ES	EWS/FLI Type III	Wild-type	Wild-type	Wild-type	0.67 ± 0.02	0.810 ± 0.126	300.14 ± 43.82
RD-ES	ES	EWS/FLI Type II	R273C	Null exp	Wild-type	5.66 ± 0.75	0.857 ± 0.068	ND
EWS-502	ES	EWS/FLI Type I	C135F	Null exp	Wild-type	4.99 ± 0.17	0.902 ± 0.078	>330
CHLA-10	ES	EWS/FLI Type I	NF	UN	UN	2.94 ± 0.26	0.952 ± 0.060	>330
ES-2	ES	EWS/FLI Type III	R175H	E523*	UN	1.98 ± 0.22	1.128 ± 0.126	>330
TC-71	ES	EWS/FLI Type I	R213*	Wild-type	Wild-type	3.14 ± 0.50	1.593 ± 0.036	ND

hMSC	hMSC	–	UN	UN	UN	–	>4	ND
TIP5	HUFF	–	UN	UN	UN	–	18.20 ± 2.131	ND

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).