Structure-Function Based Molecular Relationships in Ewing’s Sarcoma
Table 1
Cancer-associated gene fusions in ESFTs and EWS Fusion Proteins (EFPs) related tumours. Presented are the Fusion genes (providing DNA-binding) of EAD (providing Trans-activation) in human cancer. EFPs and the tumors that they cause: Ewing’s sarcoma, Desmoplastic Small Round Cell Tumor, Myxoid Liposarcoma, Extraskeletal Myxoid Chondrosarcoma, Malignant Melanoma of Soft Parts or Clear Cell Sarcoma, Acute leukaemia. The EAD contains a similar region of EWS in each case, including minimally the first seven exons of EWS. The EWS homologue translocated in Liposarcoma (TLS) provides the EAD. EWS fusion partners provide the DNA-binding domain of the EFP.
Trans-activation
DNA-binding
Tumor
Karyotype
EAD
FLI-1
Ewing’s sarcoma family of tumours
t(11;22)(q24;q12)
EAD
ERG
Ewing’s sarcoma family of tumours
t(21;22)(q22;q12)
EAD
ETV1
Ewing’s sarcoma family of tumours
t(7;22)(p22;q12)
EAD
ETV4/E1AF
Ewing’s sarcoma family of tumours
t(17;22)(q12;q12)
EAD
FEV
Ewing’s sarcoma family of tumours
t(2;22)(q33;q12)
EAD (TLS)
CHOP
Myxoid liposarcoma
t(12;22)(q13;q12)
EAD
ATF-1
Malignant melanoma of soft parts/soft tissue clear cell sarcoma
t(12;22)(q13;q12)
EAD
WT1
Desmoplastic small round cell tumour
t(11;22)(p13;q12)
EAD
ZSG
Small round cell sarcoma Ewing’s sarcoma/PNET (rare)