Identification of a novel fusion gene involving hTAFII68 and CHN from a t(9;17)(q22;q11.2) translocation in an extraskeletal myxoid chondrosarcoma.
AffiliationCRC Department of Cancer Genetics, Paterson Institute for Cancer Research, Wilmslow Road, Manchester, UK.
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AbstractA proportion of extraskeletal myxoid chondrosarcomas (EMC) have been shown to have a characteristic translocation t(9;22)(q22;q12) involving the EWS gene at 22q12 and the CHN orphan nuclear receptor gene at 9q22. This translocation appears to be largely specific for EMC, but has not been detected in all such tumours. We report here a case of EMC with a t(9;17)(q22;q11.2) as the sole chromosome abnormality. We have determined that the translocation results in the fusion of the entire coding region of CHN to the N-terminal transactivation domain of RBP56/hTAFII68. This is the first report of a translocation involving RBP56/hTAFII 68, a protein with sequence homology to both EWS and TLS/FUS. The involvement of RBP56/hTAFII68 may explain some unusual features of the tumour.
CitationIdentification of a novel fusion gene involving hTAFII68 and CHN from a t(9;17)(q22;q11.2) translocation in an extraskeletal myxoid chondrosarcoma. 1999, 18 (52):7599-601 Oncogene
- Fusion of the RBP56 and CHN genes in extraskeletal myxoid chondrosarcomas with translocation t(9;17)(q22;q11).
- Authors: Panagopoulos I, Mencinger M, Dietrich CU, Bjerkehagen B, Saeter G, Mertens F, Mandahl N, Heim S
- Issue date: 1999 Dec 9
- Molecular genetic characterization of the EWS/CHN and RBP56/CHN fusion genes in extraskeletal myxoid chondrosarcoma.
- Authors: Panagopoulos I, Mertens F, Isaksson M, Domanski HA, Brosjö O, Heim S, Bjerkehagen B, Sciot R, Dal Cin P, Fletcher JA, Fletcher CD, Mandahl N
- Issue date: 2002 Dec
- Fusion of the EWS-related gene TAF2N to TEC in extraskeletal myxoid chondrosarcoma.
- Authors: Sjögren H, Meis-Kindblom J, Kindblom LG, Aman P, Stenman G
- Issue date: 1999 Oct 15
- Fusion of the NH2-terminal domain of the basic helix-loop-helix protein TCF12 to TEC in extraskeletal myxoid chondrosarcoma with translocation t(9;15)(q22;q21).
- Authors: Sjögren H, Wedell B, Meis-Kindblom JM, Kindblom LG, Stenman G
- Issue date: 2000 Dec 15
- Extraskeletal myxoid chondrosarcoma: a clinicopathologic, immunohistochemical, and molecular analysis of 18 cases.
- Authors: Okamoto S, Hisaoka M, Ishida T, Imamura T, Kanda H, Shimajiri S, Hashimoto H
- Issue date: 2001 Oct