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ABL-BCR expression does not correlate with deletions on the derivative chromosome 9 or survival in chronic myeloid leukemia

Chronic myeloid leukemia (CML) is caused by a t(9;22)(q34;q11) reciprocal translocation that generates a BCR-ABLfusion gene on the 22q- or Philadelphia (Ph) chromosome and anABL-BCR gene on 9q+.12 Large deletions adjacent to the t(9;22) breakpoint on the derivative 9q+ chromosome have now been found...

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Main Authors: Fuente, Josu de la (Author) , Merx, Kirsten (Author) , Steer, E. Joanna (Author) , Müller, Martin (Author) , Szydlo, Richard M. (Author) , Maywald, Ole (Author) , Berger, Ute (Author) , Hehlmann, Rüdiger (Author) , Goldman, John M. (Author) , Cross, Nicholas C. P. (Author) , Melo, Junia V. (Author) , Hochhaus, Andreas (Author)
Format: Article (Journal)
Language:English
Published: November 1, 2001
In: Blood
Year: 2001, Volume: 98, Issue: 9, Pages: 2879-2880
ISSN:1528-0020
DOI:10.1182/blood.V98.9.2879
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1182/blood.V98.9.2879
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Author Notes:Josu de la Fuente, Kirsten Merx, E. Joanna Steer, Martin Müller, Richard M. Szydlo, Ole Maywald, Ute Berger, Rüdiger Hehlmann, John M. Goldman, Nicholas C. P. Cross, Junia V. Melo, Andreas Hochhaus, and the German CML Study Group
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Summary:Chronic myeloid leukemia (CML) is caused by a t(9;22)(q34;q11) reciprocal translocation that generates a BCR-ABLfusion gene on the 22q- or Philadelphia (Ph) chromosome and anABL-BCR gene on 9q+.12 Large deletions adjacent to the t(9;22) breakpoint on the derivative 9q+ chromosome have now been found, which result in genomic loss at both sides of the translocation breakpoint.3456 Recently, Huntly et al7 analyzed a large series of CML patients and confirmed that these deletions identify a subgroup of patients with relatively poor prognosis. However, the molecular basis of this survival disadvantage has not been identified. Due to its unique location spanning the chromosome 9/chromosome 22 junction, ABL-BCRappears as the most likely candidate for this role for the following reasons: (1) it occupies the minimal region of deletion in the reported cases6; (2) it is a “single-allele” gene whose deletion would result directly in total absence of the gene product, without a need to invoke a second event in a “2-hit” model8; and (3) it is known not to be expressed in approximately one third of CML patients,29 a frequency similar to that found for the 9q+ deletions.6
Item Description:Gesehen am 18.05.2022
Physical Description:Online Resource
ISSN:1528-0020
DOI:10.1182/blood.V98.9.2879

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