TY - JOUR
T1 - BCR/ABL genes and leukemic phenotype
T2 - From molecular mechanisms to clinical correlations
AU - Pane, Fabrizio
AU - Intrieri, Mariano
AU - Quintarelli, Concetta
AU - Izzo, Barbara
AU - Muccioli, Giada Casadei
AU - Salvatore, Francesco
PY - 2002/12/9
Y1 - 2002/12/9
N2 - The Philadelphia chromosome (Ph), a minute chromosome that derives from the balanced translocation between chromosomes 9 and 22, was first described in 1960 and was for a long time the only genetic lesion consistently associated with human cancer. This chromosomal translocation results in the fusion between the 5′ part of BCR gene, normally located on chromosome 22, and the 3′ part of the ABL gene on chromosome 9 giving origin to a BCR/ABL fusion gene which is transcribed and then translated into a hybrid protein. Three main variants of the BCR/ABL gene have been described, that, depending on the length of the sequence of the BCR gene included, encode for the p190BCR/ABL, P210BCR/ABL, and P230BCR/ABL proteins. These three main variants are associated with distinct clinical types of human leukemias. Herein we review the data on the correlations between the type of BCR/ABL gene and the corresponding leukemic clinical features. Lastly, drawing on experimental data, we provide insight into the different transforming power of the three hybrid BCR/ABL proteins.
AB - The Philadelphia chromosome (Ph), a minute chromosome that derives from the balanced translocation between chromosomes 9 and 22, was first described in 1960 and was for a long time the only genetic lesion consistently associated with human cancer. This chromosomal translocation results in the fusion between the 5′ part of BCR gene, normally located on chromosome 22, and the 3′ part of the ABL gene on chromosome 9 giving origin to a BCR/ABL fusion gene which is transcribed and then translated into a hybrid protein. Three main variants of the BCR/ABL gene have been described, that, depending on the length of the sequence of the BCR gene included, encode for the p190BCR/ABL, P210BCR/ABL, and P230BCR/ABL proteins. These three main variants are associated with distinct clinical types of human leukemias. Herein we review the data on the correlations between the type of BCR/ABL gene and the corresponding leukemic clinical features. Lastly, drawing on experimental data, we provide insight into the different transforming power of the three hybrid BCR/ABL proteins.
KW - Molecular pathogenesis
KW - Ph positive leukemias
KW - Phenotype of leukemia
KW - Philadelphia chromosome
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U2 - 10.1038/sj.onc.1206194
DO - 10.1038/sj.onc.1206194
M3 - Article
C2 - 12476311
AN - SCOPUS:0037049764
VL - 21
SP - 8652
EP - 8667
JO - Oncogene
JF - Oncogene
SN - 0950-9232
IS - 56 REV. ISS. 7
ER -