Document Detail

Identification of PML as novel PAX5 fusion partner in childhood acute lymphoblastic leukaemia.
MedLine Citation:
PMID:  17897302     Owner:  NLM     Status:  MEDLINE    
PAX5 encodes the B-cell lineage specific activator protein (BSAP) and is required for B-cell development and maintenance. In B-cell precursor acute lymphoblastic leukaemia (ALL), PAX5 is involved in several chromosome translocations that fuse the N-terminal paired DNA-binding domain of PAX5 with the C-terminal regulatory sequences of ETV6, FOXP1, ZNF521 or ELN. Herein, we describe the identification of a novel recurrent t(9;15)(p13;q24) in two cases of childhood ALL, which results in an in-frame fusion of PAX5 to the promyelocytic leukaemia (PML) gene. The putative PAX5-PML fusion gene encodes a chimaeric protein that retains the paired domain, the octapeptid and the partial homeodomain of PAX5, and virtually the whole PML protein. The steadily increasing number of PAX5 rearrangements suggests that PAX5 is not only crucial for B-cell lymphopoiesis but also for the development of B-cell malignancies.
Karin Nebral; Margit König; Lana Harder; Reiner Siebert; Oskar A Haas; Sabine Strehl
Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  British journal of haematology     Volume:  139     ISSN:  0007-1048     ISO Abbreviation:  Br. J. Haematol.     Publication Date:  2007 Oct 
Date Detail:
Created Date:  2007-09-27     Completed Date:  2008-02-21     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0372544     Medline TA:  Br J Haematol     Country:  England    
Other Details:
Languages:  eng     Pagination:  269-74     Citation Subset:  IM    
CCRI, Children's Cancer Research Institute, St. Anna Kinderkrebsforschung, Vienna, Austria.
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MeSH Terms
Amino Acid Sequence
B-Cell-Specific Activator Protein / genetics*
Base Sequence
Chromosomes, Human, Pair 15
Chromosomes, Human, Pair 9
In Situ Hybridization, Fluorescence
Molecular Sequence Data
Neoplasm Proteins / genetics*
Nuclear Proteins / genetics*
Oncogene Proteins, Fusion / genetics*
Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics*
Reverse Transcriptase Polymerase Chain Reaction
Transcription Factors / genetics*
Translocation, Genetic
Tumor Suppressor Proteins / genetics*
Reg. No./Substance:
0/B-Cell-Specific Activator Protein; 0/Neoplasm Proteins; 0/Nuclear Proteins; 0/Oncogene Proteins, Fusion; 0/PAX5 protein, human; 0/Transcription Factors; 0/Tumor Suppressor Proteins; 143220-95-5/PML protein, human

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