{
  "abstract": "Alternative lengthening of telomeres (ALT) is a telomere elongation mechanism activated during oncogenesis and primarily acting in tumors of mesenchymal origin. Although the proteins involved in the machinery enabling ALT to elongate telomeres are becoming better understood, the underlying biology of this mechanism remains unclear. In the present study, we took advantage of a fully characterized cohort of 98 leiomyosarcomas (LMS) from the French Sarcoma Group to further our understanding of the ALT mechanism. We first compared the transcriptomic profiles of ALT+ and TERT+ LMS and demonstrated a strong enrichment of the CINSARC signature in ALT+ tumors. The establishment of an ALT+-related signature in these LMS confirmed the close association between CINSARC and ALT in two additional cohorts of non-translocation-related sarcomas. In vitro mesenchymal models of spontaneous ALT induction showed increased CINSARC expression following acquisition of the ALT mechanism. Conversely, ALT inactivation, through BLM inhibition, led to decreased CINSARC expression. These results establish CINSARC as a new hallmark of the ALT mechanism in non-translocation-related sarcomas and demonstrate the association of a cellular biological process with the CINSARC prognostic signature, namely the ALT mechanism.\n\nSupplementary Information: The online version contains supplementary material available at https://doi.org/10.1186/s12885-026-16151-7.",
  "abstract_shown": true,
  "affiliations": [
    "Univ Toulouse, CNRS, Inserm, CRCT, 2 Avenue Hubert Curien, Toulouse, Cedex 1, 31037 France",
    "Centre Hospitalier Universitaire (CHU) de Toulouse, IUCT‐Oncopole, Toulouse, France",
    "Department of Pathology, Groupe Hospitalier Est, Hospices Civils de Lyon, Bron, France",
    "Pathophysiology and Genetics of Neuron and Muscle (PGNM), Institut Neuro Myo Gène (INMG), Université Claude Bernard Lyon 1, CNRS UMR 5261-INSERM U1315, Neuron-Muscle Interaction Team, Lyon, France",
    "PhyMedExp, Inserm, University of Montpellier, CNRS, Montpellier, France",
    "Cancer Research Center of Lyon, Centre Léon Bérard, University Claude Bernard Lyon 1, INSERM 1052, CNRS 5286, Lyon, France",
    "Department of Pathology, Oncopole Claudius Régaud, IUCT‐Oncopole, Toulouse, France"
  ],
  "article_type": "research article",
  "arxiv": null,
  "author_total": 9,
  "authors": [
    {
      "affs": [
        0,
        1
      ],
      "name": "Gaëlle Pérot"
    },
    {
      "affs": [
        2,
        3
      ],
      "name": "Clémence Guerriau"
    },
    {
      "affs": [
        0
      ],
      "name": "Natacha Roussel"
    },
    {
      "affs": [
        0
      ],
      "name": "Emeline Sarot"
    },
    {
      "affs": [
        0
      ],
      "name": "Carine Valle"
    },
    {
      "affs": [
        4
      ],
      "name": "Pascal Pomiès"
    },
    {
      "affs": [
        5
      ],
      "name": "Franck Tirode"
    },
    {
      "affs": [
        2,
        3
      ],
      "name": "Delphine Poncet"
    },
    {
      "affs": [
        0,
        6
      ],
      "name": "Frédéric Chibon"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/9ehmqx9kmxtfq.bib",
  "cited_by": 1,
  "doi": "10.1186/s12885-026-16151-7",
  "fulltext_url": "https://pmc.ncbi.nlm.nih.gov/articles/PMC13718010/",
  "id": "9ehmqx9kmxtfq",
  "issns": [
    "1471-2407"
  ],
  "landing_url": "https://doi.org/10.1186/s12885-026-16151-7",
  "language": "en",
  "licence": "CC-BY-NC-ND-4.0",
  "licence_label": "CC BY-NC-ND 4.0",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by-nc-nd/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "heading",
      "Research"
    ],
    [
      "openalex",
      "2737|Telomeres, Telomerase, and Senescence"
    ]
  ],
  "openalex": "W7164001032",
  "pdf_url": null,
  "pmcid": "PMC13718010",
  "pmid": "42265633",
  "published": "2026-06-09",
  "publisher": "BMC",
  "ris": "https://research.draconitic.com/paper/9ehmqx9kmxtfq.ris",
  "sources": [
    {
      "first_seen": "2026-11-02T12:22:10Z",
      "native_id": "PMC13718010",
      "source": "pmc",
      "url": "https://doi.org/10.1186/s12885-026-16151-7"
    }
  ],
  "subjects": [
    {
      "field": "medicine",
      "origin": "openalex:2737|Telomeres, Telomerase, and Senescence",
      "subfield": "physiology"
    }
  ],
  "title": "Alternative lengthening of telomeres and CINSARC are interconnected toward non-translocation-related sarcomas progression",
  "title_text": "Alternative lengthening of telomeres and CINSARC are interconnected toward non-translocation-related sarcomas progression",
  "type": "peer-reviewed",
  "updated_at": "2026-11-02T12:22:10Z",
  "url": "https://research.draconitic.com/paper/9ehmqx9kmxtfq",
  "venue": "BMC Cancer",
  "versions": []
}