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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
15
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pubmed:dateCreated |
1997-8-27
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pubmed:abstractText |
Chromosomal translocations identified in hematopoietic and solid tumors result in deregulated expression of protooncogenes or creation of chimeric proteins with tumorigenic potential. In the pediatric solid tumor alveolar rhabdomyosarcoma, a consistent t(2;13)(q35;q14) or variant t(1;13)(p36;q14) translocation generates PAX3-FKHR or PAX7-FKHR fusion proteins, respectively. In this report, we demonstrate that in addition to functional alterations these translocations are associated with fusion product overexpression. Furthermore, PAX3-FKHR and PAX7-FKHR overexpression occurs by distinct mechanisms. Transcription of PAX3-FKHR is increased relative to wild-type PAX3 by a copy number-independent process. In contrast, PAX7-FKHR overexpression results from fusion gene amplification. Thus, gene-specific mechanisms were selected to overexpress PAX3-FKHR and PAX7-FKHR in alveolar rhabdomyosarcoma, presumably due to differences in regulation between the wild-type loci. We postulate that these overexpression mechanisms ensure a critical level of gene product for the oncogenic effects of these fusions.
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pubmed:grant |
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pubmed:commentsCorrections |
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-1283315,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-1283318,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-1663783,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-1736310,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-1982921,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-2022185,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-6086008,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-6499844,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-7506981,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-7530783,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-7744814,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-7782066,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-7862145,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-7969446,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-8098985,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-8162068,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-8187070,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-8221646,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-8275086,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-8643596,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-8695764,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-8789435,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-8790412,
http://linkedlifedata.com/resource/pubmed/commentcorrection/9223312-8917562
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/DNA-Binding Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/FOXO1 protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Forkhead Transcription Factors,
http://linkedlifedata.com/resource/pubmed/chemical/Homeodomain Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Muscle Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Nerve Tissue Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/PAX3 protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/PAX7 Transcription Factor,
http://linkedlifedata.com/resource/pubmed/chemical/PAX7 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/Transcription Factors
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pubmed:status |
MEDLINE
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pubmed:month |
Jul
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pubmed:issn |
0027-8424
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:day |
22
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pubmed:volume |
94
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
8047-51
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pubmed:dateRevised |
2009-11-18
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pubmed:meshHeading |
pubmed-meshheading:9223312-Chromosomes, Human, Pair 1,
pubmed-meshheading:9223312-Chromosomes, Human, Pair 13,
pubmed-meshheading:9223312-Chromosomes, Human, Pair 2,
pubmed-meshheading:9223312-Cloning, Molecular,
pubmed-meshheading:9223312-DNA-Binding Proteins,
pubmed-meshheading:9223312-Forkhead Transcription Factors,
pubmed-meshheading:9223312-Homeodomain Proteins,
pubmed-meshheading:9223312-Humans,
pubmed-meshheading:9223312-Muscle Proteins,
pubmed-meshheading:9223312-Nerve Tissue Proteins,
pubmed-meshheading:9223312-PAX7 Transcription Factor,
pubmed-meshheading:9223312-Paired Box Transcription Factors,
pubmed-meshheading:9223312-Rhabdomyosarcoma, Alveolar,
pubmed-meshheading:9223312-Transcription Factors,
pubmed-meshheading:9223312-Transcriptional Activation,
pubmed-meshheading:9223312-Translocation, Genetic,
pubmed-meshheading:9223312-Tumor Cells, Cultured
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pubmed:year |
1997
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pubmed:articleTitle |
Fusion genes resulting from alternative chromosomal translocations are overexpressed by gene-specific mechanisms in alveolar rhabdomyosarcoma.
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pubmed:affiliation |
Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 37th Street and Hamilton Walk, Philadelphia, PA 19104-6082, USA.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
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