|
rdf:type |
|
|
lifeskim:mentions |
|
|
pubmed:issue |
13
|
|
pubmed:dateCreated |
1999-8-31
|
|
pubmed:abstractText |
The Pax3-FKHR fusion protein is present in alveolar rhabdomyosarcoma and results from the t(2;13) (q35;q14) chromosomal translocation. Its oncogenic activity is dependent on a combination of protein-DNA and protein-protein interactions mediated by the Pax3 homeodomain recognition helix. In this report we demonstrate that human Daxx (hDaxx) interacts with Pax3 in vivo and with DNA-bound Pax3 in vitro. This interaction is mediated primarily through the homeodomain recognition helix with the additional involvement of the octapeptide domain and its N-terminal flanking amino acids. Through this interaction hDaxx represses the transcriptional activity of Pax3 by approximately 80%. The Pax3-FKHR fusion is unresponsive to this repressive effect despite an observed endogenous interaction with hDaxx in a rhabdomyosarcoma tumor cell line. Therefore, these data support the model that fusion of FKHR to Pax3 not only adds a strong transactivation domain, but also deregulates transcriptional control of Pax3 by overriding the natural repressive effect of hDaxx.
|
|
pubmed:grant |
|
|
pubmed:language |
eng
|
|
pubmed:journal |
|
|
pubmed:citationSubset |
IM
|
|
pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/Adaptor Proteins, Signal Transducing,
http://linkedlifedata.com/resource/pubmed/chemical/Carrier Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/DAXX protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/DNA,
http://linkedlifedata.com/resource/pubmed/chemical/DNA-Binding Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Daxx protein, mouse,
http://linkedlifedata.com/resource/pubmed/chemical/FOXO1 protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Forkhead Transcription Factors,
http://linkedlifedata.com/resource/pubmed/chemical/Intracellular Signaling Peptides...,
http://linkedlifedata.com/resource/pubmed/chemical/Nuclear Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Oncogene Proteins, Fusion,
http://linkedlifedata.com/resource/pubmed/chemical/PAX3 protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Paired Box Transcription Factors,
http://linkedlifedata.com/resource/pubmed/chemical/Pax3 protein, mouse,
http://linkedlifedata.com/resource/pubmed/chemical/Repressor Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Transcription Factors
|
|
pubmed:status |
MEDLINE
|
|
pubmed:month |
Jul
|
|
pubmed:issn |
0261-4189
|
|
pubmed:author |
|
|
pubmed:issnType |
Print
|
|
pubmed:day |
1
|
|
pubmed:volume |
18
|
|
pubmed:owner |
NLM
|
|
pubmed:authorsComplete |
Y
|
|
pubmed:pagination |
3702-11
|
|
pubmed:dateRevised |
2008-11-20
|
|
pubmed:meshHeading |
pubmed-meshheading:10393185-Adaptor Proteins, Signal Transducing,
pubmed-meshheading:10393185-Amino Acid Sequence,
pubmed-meshheading:10393185-Carrier Proteins,
pubmed-meshheading:10393185-Cell Line,
pubmed-meshheading:10393185-Cloning, Molecular,
pubmed-meshheading:10393185-DNA,
pubmed-meshheading:10393185-DNA-Binding Proteins,
pubmed-meshheading:10393185-Forkhead Transcription Factors,
pubmed-meshheading:10393185-Gene Expression Regulation, Neoplastic,
pubmed-meshheading:10393185-Humans,
pubmed-meshheading:10393185-Intracellular Signaling Peptides and Proteins,
pubmed-meshheading:10393185-Molecular Sequence Data,
pubmed-meshheading:10393185-Molecular Weight,
pubmed-meshheading:10393185-Nuclear Proteins,
pubmed-meshheading:10393185-Oncogene Proteins, Fusion,
pubmed-meshheading:10393185-Paired Box Transcription Factors,
pubmed-meshheading:10393185-Phosphorylation,
pubmed-meshheading:10393185-Precipitin Tests,
pubmed-meshheading:10393185-Protein Binding,
pubmed-meshheading:10393185-Repressor Proteins,
pubmed-meshheading:10393185-Rhabdomyosarcoma,
pubmed-meshheading:10393185-Sequence Deletion,
pubmed-meshheading:10393185-Transcription Factors,
pubmed-meshheading:10393185-Transfection,
pubmed-meshheading:10393185-Translocation, Genetic,
pubmed-meshheading:10393185-Yeasts
|
|
pubmed:year |
1999
|
|
pubmed:articleTitle |
The Pax3-FKHR oncoprotein is unresponsive to the Pax3-associated repressor hDaxx.
|
|
pubmed:affiliation |
Departments of Genetics, St Jude Children's Research Hospital, 332 North Lauderdale, Memphis, TN 38105, USA.
|
|
pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
|
