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FUSION OF SYT TO 2 GENES, SSX1 AND SSX2, ENCODING PROTEINS WITH HOMOLOGY TO THE KRUPPEL-ASSOCIATED BOX IN HUMAN SYNOVIAL SARCOMA
Author(s): CREW AJ, CLARK J, FISHER C, GILL S, GRIMER R, CHAND A, SHIPLEY J, GUSTERSON BA, COOPER CS
Source: EMBO JOURNAL    Volume: 14    Issue: 10    Pages: 2333-2340    Published: MAY 15 1995  
Times Cited: 253     References: 43     
Abstract: We demonstrate that the cytogenetically defined translocation t(X;18)(p11.2;q11.2) found in human synovial sarcoma results in the fusion of the chromosome 18 SYT gene to either of two distinct genes, SSX1 or SSX2, at Xp11.2. The SSX1 and SSX2 genes encode closely related proteins (81% identity) of 188 amino acids that are rich in charged amino acids. The N-terminal portion of each SSX protein exhibits homology to the Kruppel-associated box (KRAB), a transcriptional repressor domain previously found only in Kruppel-type zinc finger proteins. PCR analysis demonstrates the presence of SYT-SSX1 or SYT-SSX2 fusion transcripts in 29 of 32 of the synovial sarcomas examined, indicating that the detection of these hybrid transcripts by PCR may represent a very useful diagnostic method. Sequence analysis has demonstrated heterogeneity in the fusion transcripts with the formation of two distinct SYT-SSX1 fusion junctions and two distinct SYT-SSX2 fusion junctions.
Document Type: Article
Language: English
Addresses:
1. INST CANC RES, HADDOW LABS, MOLEC CARCINOGENESIS SECT, SUTTON SM2 5NG, SURREY ENGLAND
2. INST CANC RES, HADDOW LABS, CELL BIOL & EXPTL BIOL SECT, SUTTON SM2 5NG, SURREY ENGLAND
3. ROYAL MARSDEN HOSP, LONDON SW3 6JJ, ENGLAND
4. ROYAL ORTHOPAED HOSP, BIRMINGHAM B31 2AP, W MIDLANDS ENGLAND
5. UNIV OXFORD, DEPT BIOCHEM, GENET LAB, OXFORD OX1 3QU, ENGLAND
Publisher: OXFORD UNIV PRESS UNITED KINGDOM, WALTON ST JOURNALS DEPT, OXFORD, ENGLAND OX2 6DP
Subject Category: Biochemistry & Molecular Biology; Cell Biology
IDS Number: QZ862
ISSN: 0261-4189
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