Fusion of the EWS gene to CHN, a member of the steroid/thyroid receptor gene superfamily, in a human myxoid chondrosarcoma.
The specific chromosomal translocation t(9;22)(q22-31;q11-12) has been observed in the myxoid variant of human chondrosarcoma. In agreement with this observation we report that the EWS gene located at chromosome band 22q12 becomes fused to CHN, a member of the steroid/thyroid receptor gene superfamily located at 9q22-31, in a skeletal myxoid chondrosarcoma. CHN appears to be the human homologue of the rat gene NOR1, which was recently identified as a sequence overexpressed in rat brain cells undergoing apoptosis. Our results also indicate that the chimaeric EWS-CHN gene encodes a EWS-CHN fusion protein in which the C-terminal RNA-binding domain of EWS is replaced by the entire CHN protein, comprising a long N-terminal domain, a central DNA binding domain and a C-terminal ligand-binding/dimerisation domain.
Clark
J
J
Section of Cell Biology and Experimental Pathology, Haddow Laboratories, Belmont, Sutton, Surrey, UK.
Benjamin
H
H
Gill
S
S
Sidhar
S
S
Goodwin
G
G
Crew
J
J
Gusterson
B A
BA
Shipley
J
J
Cooper
C S
CS
D000595:Amino Acid Sequence; D000818:Animals; D001483:Base Sequence; D002813:Chondrosarcoma / Q000235:genetics*; D003001:Cloning, Molecular; D004247:DNA / Q000378:metabolism; D006801:Humans; D008969:Molecular Sequence Data; D009857:Oncogenes; D016133:Polymerase Chain Reaction; D051381:Rats; D011987:Receptors, Steroid / Q000235:genetics*; D011988:Receptors, Thyroid Hormone / Q000235:genetics*; D012512:Sarcoma, Ewing / Q000235:genetics*
D011987:Receptors, Steroid; D011988:Receptors, Thyroid Hormone; D004247:DNA