PROSITE documentation PDOC50806KRAB-related domain profile
This domain of about 63 amino acid residues is related to the KRAB domain (see <PDOC50805> and has been identified in the N-terminal ends of proteins belonging to the SSX family [1,2,3]. Proteins of the SSX family lack the C2H2-type zinc finger (see <PDOC00028>) which is invariably found in proteins containing the KRAB domain. The KRAB-related domains show greatest homology to the KRAB A subdomain and contain a high proportion of the amino acids which are usually conserved between the KRAB A subdomains of zinc finger proteins [1].
Although the KRAB-related domains of SSX1 and SSX2 contribute to the repressive activity of these proteins, transcriptional repression is mainly mediated by a region located in their C-terminus. Hence, unlike the KRAB domain, the KRAB-related domain is only a weak transcriptional repression domain. In addition the KRAB-related domain is unable to interact with the KAP-1/TIF1-β protein which appears to function as a universal corepressor for KRAB domain proteins [4].
Translocations which result in the fusion of the SYT gene to either of the closely related SSX1 or SSX2 genes are associated with synovial sarcomas [1]. The resulting SYT-SSX1/SSX2 proteins analyzed so far lack the KRAB-related domain and act as activators of transcription [4].
The profile we developed spans the complete KRAB-related domain.
Last update:December 2001 / First entry.
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PROSITE method (with tools and information) covered by this documentation:
| 1 | Authors | Crew A.J. Clark J. Fisher C. Gill S. Grimer R. Chand A. Shipley J. Gusterson B.A. Cooper C.S. |
| Title | Fusion of SYT to two genes, SSX1 and SSX2, encoding proteins with homology to the Kruppel-associated box in human synovial sarcoma. | |
| Source | EMBO J. 14:2333-2340(1995). | |
| PubMed ID | 7539744 |
| 2 | Authors | de Leeuw B. Balemans M. Geurts van Kessel A. |
| Source | Cytogenet. Cell Genet. 73:179-183(1996). |
| 3 | Authors | Gure A.O. Tureci O. Sahin U. Tsang S. Scanlan M.J. Jager E. Knuth A. Pfreundschuh M. Old L.J. Chen Y.-T. |
| Title | SSX: a multigene family with several members transcribed in normal testis and human cancer. | |
| Source | Int. J. Cancer 72:965-971(1997). | |
| PubMed ID | 9378559 | |
| DOI | 10.1002/(SICI)1097-0215(19970917)72:6<965::AID-IJC8>3.0.CO;2-N |
| 4 | Authors | Lim F.L. Soulez M. Koczan D. Thiesen H.-J. Knight J.C. |
| Title | A KRAB-related domain and a novel transcription repression domain in proteins encoded by SSX genes that are disrupted in human sarcomas. | |
| Source | Oncogene 17:2013-2018(1998). | |
| PubMed ID | 9788446 | |
| DOI | 10.1038/sj.onc.1202122 |
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