Genomic disruption of the histone methyltransferase SETD2 in chronic lymphocytic leukaemia

Histone methyltransferases (HMTs) are important epigenetic regulators of gene transcription and are disrupted at the genomic level in a spectrum of human tumours including haematological malignancies. Using high-resolution single nucleotide polymorphism (SNP) arrays, we identified recurrent deletion...

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Autores: Parker, Helen, Rose-Zerilli, M. J. J., Larráyoz, María José, Clifford, R., Edelmann, J., Blakemore, S., Gibson, J., Wang, J., Ljungström, V., Wojdacz, T. K., Chaplin, T., Roghanian, A., Davis, Zadie, Parker, Antón, Tausch, E., Ntoufa, S., Ramos, S., Robbe, P., Alsolami, R., Steele, A. J., Packham, G., Rodríguez-Vicente, Ana Eugenia, Brown, L., McNicholl, F., Forconi, F., Pettitt, A., Hillmen, P., Dyer, M., Cragg, M. S., Chelala, C., Oakes, C. C., Rosenquist, Richard, Stamatopoulos, Kostas, Stilgenbauer, S., Knight, S., Schuh, A., Oscier, David G., Strefford, Jonathan C.
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2016
País:España
Institución:Consejo Superior de Investigaciones Científicas (CSIC)
Repositorio:DIGITAL.CSIC. Repositorio Institucional del CSIC
OAI Identifier:oai:digital.csic.es:10261/168538
Acceso en línea:http://hdl.handle.net/10261/168538
Access Level:acceso abierto
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spelling Genomic disruption of the histone methyltransferase SETD2 in chronic lymphocytic leukaemiaParker, HelenRose-Zerilli, M. J. J.Larráyoz, María JoséClifford, R.Edelmann, J.Blakemore, S.Gibson, J.Wang, J.Ljungström, V.Wojdacz, T. K.Chaplin, T.Roghanian, A.Davis, ZadieParker, AntónTausch, E.Ntoufa, S.Ramos, S.Robbe, P.Alsolami, R.Steele, A. J.Packham, G.Rodríguez-Vicente, Ana EugeniaBrown, L.McNicholl, F.Forconi, F.Pettitt, A.Hillmen, P.Dyer, M.Cragg, M. S.Chelala, C.Oakes, C. C.Rosenquist, RichardStamatopoulos, KostasStilgenbauer, S.Knight, S.Schuh, A.Oscier, David G.Strefford, Jonathan C.Histone methyltransferases (HMTs) are important epigenetic regulators of gene transcription and are disrupted at the genomic level in a spectrum of human tumours including haematological malignancies. Using high-resolution single nucleotide polymorphism (SNP) arrays, we identified recurrent deletions of the SETD2 locus in 3% (8/261) of chronic lymphocytic leukaemia (CLL) patients. Further validation in two independent cohorts showed that SETD2 deletions were associated with loss of TP53, genomic complexity and chromothripsis. With next-generation sequencing we detected mutations of SETD2 in an additional 3.8% of patients (23/602). In most cases, SETD2 deletions or mutations were often observed as a clonal event and always as a mono-allelic lesion, leading to reduced mRNA expression in SETD2-disrupted cases. Patients with SETD2 abnormalities and wild-type TP53 and ATM from five clinical trials employing chemotherapy or chemo-immunotherapy had reduced progression-free and overall survival compared with cases wild type for all three genes. Consistent with its postulated role as a tumour suppressor, our data highlight SETD2 aberration as a recurrent, early loss-of-function event in CLL pathobiology linked to aggressive disease.This work was funded by Bloodwise (11052, 12036), the Kay Kendall Leukaemia Fund (873), Cancer Research UK (C34999/A18087, ECMC C24563/A15581), Wessex Medical Research and the Bournemouth Leukaemia Fund. SS is supported by the Else Kröner-FreseniusStiftung (2012_A146) and Deutsche Forschungsgemeinschaft (SFB 1074 projects B1, B2). The LRF CLL4 trial was funded by a core grant from Leukaemia and Lymphoma Research. DG and DC acknowledge the support by The Royal Marsden Hospital and The Institute of Cancer Research National Institute of Health Research Biomedical Research Center. RR is supported by the Swedish Cancer Society, the Swedish Research Council, Science for Life Laboratory, Uppsala University, Uppsala University Hospital, and the Lion’s Cancer Research Foundation, Uppsala.Peer ReviewedNature Publishing GroupLeukaemia & Lymphoma Research (UK)Royal Marsden NHS Foundation TrustThe Institute of Cancer Research (UK)Swedish Research CouncilSwedish Cancer SocietyPolysackaridforskning i Uppsala ABGerman Research FoundationLeukaemia FoundationWessex Medical ResearchKay Kendall Leukaemia FundCancer Research UKConsejo Superior de Investigaciones Científicas [https://ror.org/02gfc7t72]2018201820162018info:eu-repo/semantics/articlehttp://purl.org/coar/resource_type/c_6501Publisher's versioninfo:eu-repo/semantics/publishedVersionhttp://hdl.handle.net/10261/168538reponame:DIGITAL.CSIC. Repositorio Institucional del CSICinstname:Consejo Superior de Investigaciones Científicas (CSIC)Ingléshttps://doi.org/10.1038/leu.2016.134Síinfo:eu-repo/semantics/openAccessoai:digital.csic.es:10261/1685382026-05-22T06:33:51Z
dc.title.none.fl_str_mv Genomic disruption of the histone methyltransferase SETD2 in chronic lymphocytic leukaemia
title Genomic disruption of the histone methyltransferase SETD2 in chronic lymphocytic leukaemia
spellingShingle Genomic disruption of the histone methyltransferase SETD2 in chronic lymphocytic leukaemia
Parker, Helen
title_short Genomic disruption of the histone methyltransferase SETD2 in chronic lymphocytic leukaemia
title_full Genomic disruption of the histone methyltransferase SETD2 in chronic lymphocytic leukaemia
title_fullStr Genomic disruption of the histone methyltransferase SETD2 in chronic lymphocytic leukaemia
title_full_unstemmed Genomic disruption of the histone methyltransferase SETD2 in chronic lymphocytic leukaemia
title_sort Genomic disruption of the histone methyltransferase SETD2 in chronic lymphocytic leukaemia
dc.creator.none.fl_str_mv Parker, Helen
Rose-Zerilli, M. J. J.
Larráyoz, María José
Clifford, R.
Edelmann, J.
Blakemore, S.
Gibson, J.
Wang, J.
Ljungström, V.
Wojdacz, T. K.
Chaplin, T.
Roghanian, A.
Davis, Zadie
Parker, Antón
Tausch, E.
Ntoufa, S.
Ramos, S.
Robbe, P.
Alsolami, R.
Steele, A. J.
Packham, G.
Rodríguez-Vicente, Ana Eugenia
Brown, L.
McNicholl, F.
Forconi, F.
Pettitt, A.
Hillmen, P.
Dyer, M.
Cragg, M. S.
Chelala, C.
Oakes, C. C.
Rosenquist, Richard
Stamatopoulos, Kostas
Stilgenbauer, S.
Knight, S.
Schuh, A.
Oscier, David G.
Strefford, Jonathan C.
author Parker, Helen
author_facet Parker, Helen
Rose-Zerilli, M. J. J.
Larráyoz, María José
Clifford, R.
Edelmann, J.
Blakemore, S.
Gibson, J.
Wang, J.
Ljungström, V.
Wojdacz, T. K.
Chaplin, T.
Roghanian, A.
Davis, Zadie
Parker, Antón
Tausch, E.
Ntoufa, S.
Ramos, S.
Robbe, P.
Alsolami, R.
Steele, A. J.
Packham, G.
Rodríguez-Vicente, Ana Eugenia
Brown, L.
McNicholl, F.
Forconi, F.
Pettitt, A.
Hillmen, P.
Dyer, M.
Cragg, M. S.
Chelala, C.
Oakes, C. C.
Rosenquist, Richard
Stamatopoulos, Kostas
Stilgenbauer, S.
Knight, S.
Schuh, A.
Oscier, David G.
Strefford, Jonathan C.
author_role author
author2 Rose-Zerilli, M. J. J.
Larráyoz, María José
Clifford, R.
Edelmann, J.
Blakemore, S.
Gibson, J.
Wang, J.
Ljungström, V.
Wojdacz, T. K.
Chaplin, T.
Roghanian, A.
Davis, Zadie
Parker, Antón
Tausch, E.
Ntoufa, S.
Ramos, S.
Robbe, P.
Alsolami, R.
Steele, A. J.
Packham, G.
Rodríguez-Vicente, Ana Eugenia
Brown, L.
McNicholl, F.
Forconi, F.
Pettitt, A.
Hillmen, P.
Dyer, M.
Cragg, M. S.
Chelala, C.
Oakes, C. C.
Rosenquist, Richard
Stamatopoulos, Kostas
Stilgenbauer, S.
Knight, S.
Schuh, A.
Oscier, David G.
Strefford, Jonathan C.
author2_role author
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author
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author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
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dc.contributor.none.fl_str_mv Leukaemia & Lymphoma Research (UK)
Royal Marsden NHS Foundation Trust
The Institute of Cancer Research (UK)
Swedish Research Council
Swedish Cancer Society
Polysackaridforskning i Uppsala AB
German Research Foundation
Leukaemia Foundation
Wessex Medical Research
Kay Kendall Leukaemia Fund
Cancer Research UK
Consejo Superior de Investigaciones Científicas [https://ror.org/02gfc7t72]
description Histone methyltransferases (HMTs) are important epigenetic regulators of gene transcription and are disrupted at the genomic level in a spectrum of human tumours including haematological malignancies. Using high-resolution single nucleotide polymorphism (SNP) arrays, we identified recurrent deletions of the SETD2 locus in 3% (8/261) of chronic lymphocytic leukaemia (CLL) patients. Further validation in two independent cohorts showed that SETD2 deletions were associated with loss of TP53, genomic complexity and chromothripsis. With next-generation sequencing we detected mutations of SETD2 in an additional 3.8% of patients (23/602). In most cases, SETD2 deletions or mutations were often observed as a clonal event and always as a mono-allelic lesion, leading to reduced mRNA expression in SETD2-disrupted cases. Patients with SETD2 abnormalities and wild-type TP53 and ATM from five clinical trials employing chemotherapy or chemo-immunotherapy had reduced progression-free and overall survival compared with cases wild type for all three genes. Consistent with its postulated role as a tumour suppressor, our data highlight SETD2 aberration as a recurrent, early loss-of-function event in CLL pathobiology linked to aggressive disease.
publishDate 2016
dc.date.none.fl_str_mv 2016
2018
2018
2018
dc.type.none.fl_str_mv info:eu-repo/semantics/article
http://purl.org/coar/resource_type/c_6501
Publisher's version
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/10261/168538
url http://hdl.handle.net/10261/168538
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv https://doi.org/10.1038/leu.2016.134

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eu_rights_str_mv openAccess
dc.publisher.none.fl_str_mv Nature Publishing Group
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instname:Consejo Superior de Investigaciones Científicas (CSIC)
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