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    Nature communications. 2021 Feb 24. doi: 10.1038/s41467-021-21247-8. pii: 10.1038/s41467-021-21247-8
    Alternative lengthening of telomeres in childhood neuroblastoma from genome to proteome.
    Hartlieb SA1,  Sieverling L2,  Nadler-Holly M3,  Ziehm M4,  Toprak UH5,  Herrmann C6,  Ishaque N7,  Okonechnikov K8,  Gartlgruber M9,  Park YG10,  Wecht EM11,  Savelyeva L12,  Henrich KO13,  Rosswog C14,  Fischer M15,  Hero B16,  Jones DTW17,  Pfaff E18,  Witt O19,  Pfister SM20,  Volckmann R21,  Koster J22,  Kiesel K23,  Rippe K24,  Taschner-Mandl S25,  Ambros P26,  Brors B27,  Selbach M28,  Feuerbach L29,  Westermann F30
    Author information
    1Hopp Children's Cancer Center (KiTZ), Heidelberg, Germany.
    2Faculty of Biosciences, Heidelberg University, Heidelberg, Germany.
    3Proteome Dynamics, Max Delbrück Center for Molecular Medicine, Berlin, Germany.
    4Proteome Dynamics, Max Delbrück Center for Molecular Medicine, Berlin, Germany.
    5Hopp Children's Cancer Center (KiTZ), Heidelberg, Germany.
    6Health Data Science Unit, Medical Faculty Heidelberg and BioQuant, Heidelberg, Germany.
    7Digital Health Centre, Berlin Institute of Health (BIH), Berlin, Germany.
    8Hopp Children's Cancer Center (KiTZ), Heidelberg, Germany.
    9Hopp Children's Cancer Center (KiTZ), Heidelberg, Germany.
    10Hopp Children's Cancer Center (KiTZ), Heidelberg, Germany.
    11Hopp Children's Cancer Center (KiTZ), Heidelberg, Germany.
    12Hopp Children's Cancer Center (KiTZ), Heidelberg, Germany.
    13Hopp Children's Cancer Center (KiTZ), Heidelberg, Germany.
    14Department of Experimental Pediatric Oncology, University Children's Hospital of Cologne, Medical Faculty, Cologne, Germany.
    15Department of Experimental Pediatric Oncology, University Children's Hospital of Cologne, Medical Faculty, Cologne, Germany.
    16Department of Pediatric Oncology and Hematology, University of Cologne, Cologne, Germany.
    17Hopp Children's Cancer Center (KiTZ), Heidelberg, Germany.
    18Hopp Children's Cancer Center (KiTZ), Heidelberg, Germany.
    19Hopp Children's Cancer Center (KiTZ), Heidelberg, Germany.
    20Hopp Children's Cancer Center (KiTZ), Heidelberg, Germany.
    21Department of Oncogenomics Amsterdam University Medical Centers (AUMC), Amsterdam, the Netherlands.
    22Department of Oncogenomics Amsterdam University Medical Centers (AUMC), Amsterdam, the Netherlands.
    23Chromatin Networks, German Cancer Research Center (DKFZ) and BioQuant, Heidelberg, Germany.
    24Chromatin Networks, German Cancer Research Center (DKFZ) and BioQuant, Heidelberg, Germany.
    25CCRI, St Anna Children's Cancer Research Institute, Vienna, Austria.
    26CCRI, St Anna Children's Cancer Research Institute, Vienna, Austria.
    27Applied Bioinformatics, German Cancer Research Center (DKFZ), German Cancer Consortium (DKTK), Heidelberg, Germany.
    28Proteome Dynamics, Max Delbrück Center for Molecular Medicine, Berlin, Germany.
    29Applied Bioinformatics, German Cancer Research Center (DKFZ), German Cancer Consortium (DKTK), Heidelberg, Germany.
    30Hopp Children's Cancer Center (KiTZ), Heidelberg, Germany. f.westermann@kitz-heidelberg.de.
    Abstract

    Telomere maintenance by telomerase activation or alternative lengthening of telomeres (ALT) is a major determinant of poor outcome in neuroblastoma. Here, we screen for ALT in primary and relapsed neuroblastomas (n = 760) and characterize its features using multi-omics profiling. ALT-positive tumors are molecularly distinct from other neuroblastoma subtypes and enriched in a population-based clinical sequencing study cohort for relapsed cases. They display reduced ATRX/DAXX complex abundance, due to either ATRX mutations (55%) or low protein expression. The heterochromatic histone mark H3K9me3 recognized by ATRX is enriched at the telomeres of ALT-positive tumors. Notably, we find a high frequency of telomeric repeat loci with a neuroblastoma ALT-specific hotspot on chr1q42.2 and loss of the adjacent chromosomal segment forming a neo-telomere. ALT-positive neuroblastomas proliferate slowly, which is reflected by a protracted clinical course of disease. Nevertheless, children with an ALT-positive neuroblastoma have dismal outcome.


    Publikations ID: 33627664
    Quelle: öffnen
     
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