Projects funded by the NCN

Information on the principal investigator and host institution

Information of the project and the call

Keywords

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Toxic RNA with expanded trinucleotide repeats: pathomechanism and therapeutic targets of neurological diseases

2020/38/A/NZ3/00498

Keywords:

myotonic dystrophy fragile X syndrome fragile X tremore-ataxia syndrome neurodegeneration expansion of CUG triplet repeats expansion of CGG triplet repeats protein aggregation ribonucleoprotein complex biomolecular condensate protein sequestration alternative splicing

Descriptors:

  • NZ3_5: Molecular neurobiology
  • NZ5_3: Pathogenesis of human diseases
  • NZ2_2: Genomics, transcriptomics, epigenomics

Panel:

NZ3 - Cellular and developmental biology: cell biology, developmental biology, ageing biology, neurobiology

Host institution :

Uniwersytet im. Adama Mickiewicza w Poznaniu, Wydział Biologii

woj.

Other projects carried out by the institution 

Principal investigator (from the host institution):

prof. Krzysztof Sobczak 

Number of co-investigators in the project: 11

Call: MAESTRO 12 - announced on 2020-06-15

Amount awarded: 4 626 353 PLN

Project start date (Y-m-d): 2021-04-08

Project end date (Y-m-d): 2026-04-07

Project duration:: 60 months (the same as in the proposal)

Project status: Pending project

Project description

Download the project description in a pdf file

Note - project descriptions were prepared by the authors of the applications themselves and placed in the system in an unchanged form.

Information in the final report

  • Publication in academic press/journals (12)
  1. Distribution of alternative untranslated regions within the mRNA of the CELF1 splicing factor affects its expression.
    Authors:
    Kajdasz A, Niewiadomska D, Sekrecki M, Sobczak K.
    Academic press:
    Scientific Reports (rok: 2022, tom: 12, strony: 190), Wydawca: Nature Portfolio
    Status:
    Published
    DOI:
    10.1038/s41598-021-03901-9 - link to the publication
  2. Modulatory role of RNA helicases in MBNL-dependent alternative splicing regulation.
    Authors:
    Taylor K, Piasecka A, Kajdasz A, Brzęk A, Polay Espinoza M, Bourgeois CF, Jankowski A, Borowiak M, Raczyńska KD, Sznajder ŁJ, Sobczak K
    Academic press:
    Cellular and Molecular Life Sciences (rok: 2023, tom: 80(11), strony: 335), Wydawca: Springer
    Status:
    Published
    DOI:
    10.1007/s00018-023-04927-0 - link to the publication
  3. Sustainable recovery of MBNL activity in autoregulatory feedback loop in myotonic dystrophy
    Authors:
    Rogalska Z, Sobczak K.
    Academic press:
    Mol Ther-Nucleic Acids (rok: 2022, tom: 30, strony: 438-448), Wydawca: Springer Nature
    Status:
    Published
    DOI:
    10.023 - link to the publication
  4. Partners in crime: Proteins implicated in RNA repeat expansion diseases.
    Authors:
    Baud A, Derbis M, Tutak K, Sobczak K.
    Academic press:
    Wiley interdisciplinary reviews-RNA (rok: 2022, tom: 13, strony: e1709), Wydawca: Wiley
    Status:
    Published
    DOI:
    10.1002/wrna.1709 - link to the publication
  5. Cyclic mismatch binding ligands interact with disease-associated CGG trinucleotide repeats in RNA and suppress their translation.
    Authors:
    Konieczny P, Mukherjee S, Stepniak-Konieczna E, Taylor K, Niewiadomska D, Piasecka A, Walczak A, Baud A, Dohno C, Nakatani K, Sobczak K.
    Academic press:
    Nucleic Acids Research (rok: 2021, tom: 49, strony: 9479-9495), Wydawca: Oxford University Press
    Status:
    Published
    DOI:
    10.1093/nar/gkab669 - link to the publication
  6. Partners in crime: Proteins implicated in RNA repeat expansion diseases.
    Authors:
    Baud A, Derbis M, Tutak K, Sobczak K.
    Academic press:
    Wiley interdisciplinary reviews-RNA (rok: 2022, tom: 13, strony: e1709), Wydawca: Wiley
    Status:
    Published
    DOI:
    10.1002/wrna.1709 - link to the publication
  7. Distribution of alternative untranslated regions within the mRNA of the CELF1 splicing factor affects its expression.
    Authors:
    Kajdasz A, Niewiadomska D, Sekrecki M, Sobczak K.
    Academic press:
    Scientific Reports (rok: 2022, tom: 12, strony: 190), Wydawca: Nature Portfolio
    Status:
    Published
    DOI:
    10.1038/s41598-021-03901-9 - link to the publication
  8. Partners in crime: Proteins implicated in RNA repeat expansion diseases.
    Authors:
    Baud A, Derbis M, Tutak K, Sobczak K.
    Academic press:
    Wiley interdisciplinary reviews-RNA (rok: 2022, tom: 13, strony: e1709), Wydawca: Wiley
    Status:
    Published
    DOI:
    10.1002/wrna.1709 - link to the publication
  9. Cyclic mismatch binding ligands interact with disease-associated CGG trinucleotide repeats in RNA and suppress their translation.
    Authors:
    Konieczny P, Mukherjee S, Stepniak-Konieczna E, Taylor K, Niewiadomska D, Piasecka A, Walczak A, Baud A, Dohno C, Nakatani K, Sobczak K.
    Academic press:
    Nucleic Acids Research (rok: 2021, tom: 49, strony: 9479-9495), Wydawca: Oxford University Press
    Status:
    Published
    DOI:
    10.1093/nar/gkab669 - link to the publication
  10. Distribution of alternative untranslated regions within the mRNA of the CELF1 splicing factor affects its expression.
    Authors:
    Kajdasz A, Niewiadomska D, Sekrecki M, Sobczak K.
    Academic press:
    Scientific Reports (rok: 2022, tom: 12, strony: 190), Wydawca: Nature Portfolio
    Status:
    Published
    DOI:
    10.1038/s41598-021-03901-9 - link to the publication
  11. Sustainable recovery of MBNL activity in autoregulatory feedback loop in myotonic dystrophy
    Authors:
    Rogalska Z, Sobczak K.
    Academic press:
    Mol Ther-Nucleic Acids (rok: 2022, tom: 30, strony: 438-448), Wydawca: Springer Nature
    Status:
    Published
    DOI:
    10.023 - link to the publication
  12. Cyclic mismatch binding ligands interact with disease-associated CGG trinucleotide repeats in RNA and suppress their translation.
    Authors:
    Konieczny P, Mukherjee S, Stepniak-Konieczna E, Taylor K, Niewiadomska D, Piasecka A, Walczak A, Baud A, Dohno C, Nakatani K, Sobczak K.
    Academic press:
    Nucleic Acids Research (rok: 2021, tom: 49, strony: 9479-9495), Wydawca: Oxford University Press
    Status:
    Published
    DOI:
    10.1093/nar/gkab669 - link to the publication

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