rdf:type |
|
lifeskim:mentions |
umls-concept:C0023452,
umls-concept:C0030705,
umls-concept:C0033325,
umls-concept:C0205214,
umls-concept:C0215508,
umls-concept:C0221198,
umls-concept:C0314603,
umls-concept:C0332466,
umls-concept:C0678226,
umls-concept:C0796520,
umls-concept:C1335654,
umls-concept:C1515021,
umls-concept:C1551022,
umls-concept:C1705326,
umls-concept:C1961136
|
pubmed:issue |
12
|
pubmed:dateCreated |
1996-5-29
|
pubmed:abstractText |
The t(12;21)(p13;q22) is identified by routine cytogenetics in less than 0.05% of pediatric acute lymphoblastic leukemia (ALL) patients. This translocation encodes a TEL/AML-1 chimeric product comprising the helix-loop-helix domain of TEL, a member of the ETS-like family of transcription factors, fused to AML-1, the DNA-binding subunit of the AML-1/CBF beta transcription factor complex. Both TEL and AML-1 are involved in several myeloid leukemia-associated translocations with AML-1/CBF beta being altered in 20-30% of de novo acute myeloid leukemia (AML) cases. We now demonstrate that a TEL/AML1 chimeric transcript encoded by a cryptic t(12;21) is observed in 22% of pediatric ALL, making it the most common genetic lesion in these patients. Moreover, TEL/AML1 expression defined a distinct subgroup of patients characterized by an age between 1 and 10 years, B lineage immunophenotype, non-hyperdiploid DNA content and an excellent prognosis. These data demonstrate that molecular diagnostic approaches are invaluable in identifying clinically distinct subgroups, and that the AML1/CBF beta transcription complex is the most frequent target of chromosomal rearrangements in human leukemia.
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pubmed:grant |
|
pubmed:language |
eng
|
pubmed:journal |
|
pubmed:citationSubset |
IM
|
pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/Core Binding Factor Alpha 2 Subunit,
http://linkedlifedata.com/resource/pubmed/chemical/DNA-Binding Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/ETS translocation variant 6 protein,
http://linkedlifedata.com/resource/pubmed/chemical/Neoplasm Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Proto-Oncogene Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Proto-Oncogene Proteins c-ets,
http://linkedlifedata.com/resource/pubmed/chemical/RUNX1 protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Recombinant Fusion Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Repressor Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Transcription Factors
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pubmed:status |
MEDLINE
|
pubmed:month |
Dec
|
pubmed:issn |
0887-6924
|
pubmed:author |
|
pubmed:issnType |
Print
|
pubmed:volume |
9
|
pubmed:owner |
NLM
|
pubmed:authorsComplete |
Y
|
pubmed:pagination |
1985-9
|
pubmed:dateRevised |
2007-11-15
|
pubmed:meshHeading |
pubmed-meshheading:8609706-Base Sequence,
pubmed-meshheading:8609706-Child,
pubmed-meshheading:8609706-Child, Preschool,
pubmed-meshheading:8609706-Chromosomes, Human, Pair 12,
pubmed-meshheading:8609706-Chromosomes, Human, Pair 21,
pubmed-meshheading:8609706-Core Binding Factor Alpha 2 Subunit,
pubmed-meshheading:8609706-DNA-Binding Proteins,
pubmed-meshheading:8609706-Humans,
pubmed-meshheading:8609706-Molecular Sequence Data,
pubmed-meshheading:8609706-Neoplasm Proteins,
pubmed-meshheading:8609706-Precursor Cell Lymphoblastic Leukemia-Lymphoma,
pubmed-meshheading:8609706-Prognosis,
pubmed-meshheading:8609706-Proto-Oncogene Proteins,
pubmed-meshheading:8609706-Proto-Oncogene Proteins c-ets,
pubmed-meshheading:8609706-Recombinant Fusion Proteins,
pubmed-meshheading:8609706-Repressor Proteins,
pubmed-meshheading:8609706-Transcription Factors,
pubmed-meshheading:8609706-Translocation, Genetic
|
pubmed:year |
1995
|
pubmed:articleTitle |
TEL/AML1 fusion resulting from a cryptic t(12;21) is the most common genetic lesion in pediatric ALL and defines a subgroup of patients with an excellent prognosis.
|
pubmed:affiliation |
Department of Pathology and Laboratory Medicine, St Jude Children's Research Hospital, Memphis, TN, USA.
|
pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
|