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The Philadelphia chromosome in leukemogenesis

The truncated chromosome 22 that results from the reciprocal translocation t(9;22)(q34;q11) is known as the Philadelphia chromosome (Ph) and is a hallmark of chronic myeloid leukemia (CML). In leukemia cells, Ph not only impairs the physiological signaling pathways but also disrupts genomic stabilit...

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Autores principales: Kang, Zhi-Jie, Liu, Yu-Fei, Xu, Ling-Zhi, Long, Zi-Jie, Huang, Dan, Yang, Ya, Liu, Bing, Feng, Jiu-Xing, Pan, Yu-Jia, Yan, Jin-Song, Liu, Quentin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4896164/
https://www.ncbi.nlm.nih.gov/pubmed/27233483
http://dx.doi.org/10.1186/s40880-016-0108-0
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author Kang, Zhi-Jie
Liu, Yu-Fei
Xu, Ling-Zhi
Long, Zi-Jie
Huang, Dan
Yang, Ya
Liu, Bing
Feng, Jiu-Xing
Pan, Yu-Jia
Yan, Jin-Song
Liu, Quentin
author_facet Kang, Zhi-Jie
Liu, Yu-Fei
Xu, Ling-Zhi
Long, Zi-Jie
Huang, Dan
Yang, Ya
Liu, Bing
Feng, Jiu-Xing
Pan, Yu-Jia
Yan, Jin-Song
Liu, Quentin
author_sort Kang, Zhi-Jie
collection PubMed
description The truncated chromosome 22 that results from the reciprocal translocation t(9;22)(q34;q11) is known as the Philadelphia chromosome (Ph) and is a hallmark of chronic myeloid leukemia (CML). In leukemia cells, Ph not only impairs the physiological signaling pathways but also disrupts genomic stability. This aberrant fusion gene encodes the breakpoint cluster region-proto-oncogene tyrosine-protein kinase (BCR-ABL1) oncogenic protein with persistently enhanced tyrosine kinase activity. The kinase activity is responsible for maintaining proliferation, inhibiting differentiation, and conferring resistance to cell death. During the progression of CML from the chronic phase to the accelerated phase and then to the blast phase, the expression patterns of different BCR-ABL1 transcripts vary. Each BCR-ABL1 transcript is present in a distinct leukemia phenotype, which predicts both response to therapy and clinical outcome. Besides CML, the Ph is found in acute lymphoblastic leukemia, acute myeloid leukemia, and mixed-phenotype acute leukemia. Here, we provide an overview of the clinical presentation and cellular biology of different phenotypes of Ph-positive leukemia and highlight key findings regarding leukemogenesis.
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spelling pubmed-48961642016-06-10 The Philadelphia chromosome in leukemogenesis Kang, Zhi-Jie Liu, Yu-Fei Xu, Ling-Zhi Long, Zi-Jie Huang, Dan Yang, Ya Liu, Bing Feng, Jiu-Xing Pan, Yu-Jia Yan, Jin-Song Liu, Quentin Chin J Cancer Review The truncated chromosome 22 that results from the reciprocal translocation t(9;22)(q34;q11) is known as the Philadelphia chromosome (Ph) and is a hallmark of chronic myeloid leukemia (CML). In leukemia cells, Ph not only impairs the physiological signaling pathways but also disrupts genomic stability. This aberrant fusion gene encodes the breakpoint cluster region-proto-oncogene tyrosine-protein kinase (BCR-ABL1) oncogenic protein with persistently enhanced tyrosine kinase activity. The kinase activity is responsible for maintaining proliferation, inhibiting differentiation, and conferring resistance to cell death. During the progression of CML from the chronic phase to the accelerated phase and then to the blast phase, the expression patterns of different BCR-ABL1 transcripts vary. Each BCR-ABL1 transcript is present in a distinct leukemia phenotype, which predicts both response to therapy and clinical outcome. Besides CML, the Ph is found in acute lymphoblastic leukemia, acute myeloid leukemia, and mixed-phenotype acute leukemia. Here, we provide an overview of the clinical presentation and cellular biology of different phenotypes of Ph-positive leukemia and highlight key findings regarding leukemogenesis. BioMed Central 2016-05-27 /pmc/articles/PMC4896164/ /pubmed/27233483 http://dx.doi.org/10.1186/s40880-016-0108-0 Text en © The Author(s) 2016 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Review
Kang, Zhi-Jie
Liu, Yu-Fei
Xu, Ling-Zhi
Long, Zi-Jie
Huang, Dan
Yang, Ya
Liu, Bing
Feng, Jiu-Xing
Pan, Yu-Jia
Yan, Jin-Song
Liu, Quentin
The Philadelphia chromosome in leukemogenesis
title The Philadelphia chromosome in leukemogenesis
title_full The Philadelphia chromosome in leukemogenesis
title_fullStr The Philadelphia chromosome in leukemogenesis
title_full_unstemmed The Philadelphia chromosome in leukemogenesis
title_short The Philadelphia chromosome in leukemogenesis
title_sort philadelphia chromosome in leukemogenesis
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4896164/
https://www.ncbi.nlm.nih.gov/pubmed/27233483
http://dx.doi.org/10.1186/s40880-016-0108-0
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