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Pathogenic PPM1D variants and their impact on DNA replication

Public support

  • Provider

    Czech Science Foundation

  • Programme

    Standard projects

  • Call for proposals

    SGA0202600001

  • Main participants

    Ústav molekulární genetiky AV ČR, v. v. i.

  • Contest type

    VS - Public tender

  • Contract ID

    26-21896S

Alternative language

  • Project name in Czech

    Pathogenic PPM1D variants and their impact on DNA replication

  • Annotation in Czech

    Upon DNA damage, cells protect genome integrity by activating ATM/ATR kinases that are master regulators of the coordinated recruitment of the repair factors to DNA lesions and of the cell cycle checkpoint. PPM1D phosphatase counteracts ATM/ATR activity at chromatin and terminates the checkpoint by inhibiting the tumor suppressor p53. We have recently identified gain-of-function mutations in the PPM1D that allow proliferation in the presence of DNA damage, promote genome instability and cellular transformation. How the C-terminally truncated PPM1D mutants escape the protein degradation remains currently unclear. Here, we aim to elucidate the mechanism of the rapid turnover of PPM1D in normal cells by the proteomic analysis of the interacting proteins with the C-terminal degron. Further, we will determine how the increased PPM1D activity affects DNA replication by targeting proteins directly at the replication forks and indirectly by suppressing p53 and other signaling pathways. This project will contribute to understanding the role of PPM1D in genomic instability and cancer.

Scientific branches

  • R&D category

    ZV - Basic research

  • OECD FORD - main branch

    10608 - Biochemistry and molecular biology

  • OECD FORD - secondary branch

  • OECD FORD - another secondary branch

  • CEP - equivalent branches <br>(according to the <a href="http://www.vyzkum.cz/storage/att/E6EF7938F0E854BAE520AC119FB22E8D/Prevodnik_oboru_Frascati.pdf">converter</a>)

    CE - Biochemistry<br>EB - Genetics and molecular biology

Solution timeline

  • Realization period - beginning

    Jan 1, 2026

  • Realization period - end

    Dec 31, 2028

  • Project status

    Z - Beginning multi-year project

  • Latest support payment

Data delivery to CEP

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

  • Data delivery code

    CEP26-GA0-GA-R

  • Data delivery date

    Apr 27, 2026

Finance

  • Total approved costs

    9,357 thou. CZK

  • Public financial support

    9,024 thou. CZK

  • Other public sources

    333 thou. CZK

  • Non public and foreign sources

    0 thou. CZK