ATM is usually rearranged in T-cell prolymphocytic leukaemia (original) (raw)

Oncogene volume 16, pages 789–796 (1998)Cite this article

An Erratum to this article was published on 09 June 1998

Abstract

T-prolymphocytic leukaemia (T-PLL) is a rare, sporadic leukaemia similar to a mature T-cell leukaemia seen in some patients with Ataxia Telangiectasia (A-T), a recessive multisystem disorder caused by mutations of the ATM gene at chromosome 11q23. ATM sequence mutations have been reported in 46% of T-PLL cases, but some cases also have karyotypic abnormalities at 11q, including 11q23. This led us to investigate the structure of the ATM locus in a panel of eight cases, two of which had 11q23 abnormalities. As expected, nucleotide changes were detected in some samples. Two remission samples were wild type. To test for structural lesions, DNA fibres were hybridized with a contig of four labelled cosmids spanning the ATM locus. In all samples there were structural lesions and in four samples both alleles were affected. This provides strong evidence for our suggestion that ATM acts as a tumour suppressor during T-PLL tumorigenesis. Some additional role for ATM during T-PLL tumorigenesis is possible since nucleotide changes were present in addition to structural lesions disrupting both alleles. The mechanism of inactivation appeared to be unusual because multiple structural lesions on one allele were often observed.

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Author notes

  1. MAR Yuille and LJA Coignet: These authors contributed equally to this work

Authors and Affiliations

  1. Academic Department of Haematology and Cytogenetics, Institute of Cancer Research–Royal Marsden Hospital NHS Trust, London, SW3, UK
    MAR Yuille, LJA Coignet, SM Abraham, F Yaqub, E Matutes, V Brito-Babapulle, MJS Dyer & D Catovsky
  2. Department of Biosciences at NOVUM, Karolinska Institute, 14157, Huddinge, Sweden
    L Luo & I Vořechovský

Authors

  1. MAR Yuille
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  2. LJA Coignet
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  3. SM Abraham
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  4. F Yaqub
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  5. L Luo
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  6. E Matutes
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  7. V Brito-Babapulle
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  8. I Vořechovský
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  9. MJS Dyer
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  10. D Catovsky
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Yuille, M., Coignet, L., Abraham, S. et al. ATM is usually rearranged in T-cell prolymphocytic leukaemia.Oncogene 16, 789–796 (1998). https://doi.org/10.1038/sj.onc.1201603

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