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- Jaako, PekkaLund University,Lunds universitet,Avdelningen för molekylär hematologi,Institutionen för laboratoriemedicin,Medicinska fakulteten,Avdelningen för molekylärmedicin och genterapi,Division of Molecular Hematology (DMH),Department of Laboratory Medicine,Faculty of Medicine,Division of Molecular Medicine and Gene Therapy (author)
Disruption of the 5S RNP-Mdm2 interaction significantly improves the erythroid defect in a mouse model for Diamond-Blackfan anemia.
- Article/chapterEnglish2015
Publisher, publication year, extent ...
- 2015-05-19
- Springer Science and Business Media LLC,2015
- electronicrdacarrier
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- LIBRIS-ID:oai:lup.lub.lu.se:68bbf8ea-579a-4f7e-a781-dd1c0b0dc886
- https://lup.lub.lu.se/record/5448846URI
- https://doi.org/10.1038/leu.2015.128DOI
- http://kipublications.ki.se/Default.aspx?queryparsed=id:132393491URI
Supplementary language notes
- Language:English
- Summary in:English
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Notes
- Diamond-Blackfan anemia (DBA) is a congenital erythroid hypoplasia caused by haploinsufficiency of genes encoding ribosomal proteins (RPs). Perturbed ribosome biogenesis in DBA has been shown to induce a p53-mediated ribosomal stress response. However, the mechanisms of p53 activation and its relevance for the erythroid defect remain elusive. Previous studies have indicated that activation of p53 is caused by the inhibition of Mdm2, the main negative regulator of p53, by the 5S ribonucleoprotein particle (RNP). Meanwhile, it is not clear whether this mechanism solely mediates the p53-dependent component found in DBA. To approach this question, we crossed our mouse model for RPS19-deficient DBA with Mdm2(C305F) knock-in mice that have a disrupted 5S RNP-Mdm2 interaction. Upon induction of the Rps19 deficiency, Mdm2(C305F) reversed the p53 response and improved expansion of hematopoietic progenitors in vitro, and ameliorated the anemia in vivo. Unexpectedly, disruption of the 5S RNP-Mdm2 interaction also led to selective defect in erythropoiesis. Our findings highlight the sensitivity of erythroid progenitor cells to aberrations in p53 homeostasis mediated by the 5S RNP-Mdm2 interaction. Finally, we provide evidence indicating that physiological activation of the 5S RNP-Mdm2-p53 pathway may contribute to functional decline of the hematopoietic system in a cell-autonomous manner over time.Leukemia accepted article preview online, 19 May 2015. doi:10.1038/leu.2015.128.
Subject headings and genre
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- Debnath, ShubhranshuLund University,Lunds universitet,Avdelningen för molekylärmedicin och genterapi,Institutionen för laboratoriemedicin,Medicinska fakulteten,Division of Molecular Medicine and Gene Therapy,Department of Laboratory Medicine,Faculty of Medicine(Swepub:lu)med-sdt (author)
- Olsson, KarinLund University,Lunds universitet,Avdelningen för molekylär hematologi,Institutionen för laboratoriemedicin,Medicinska fakulteten,Avdelningen för molekylärmedicin och genterapi,Division of Molecular Hematology (DMH),Department of Laboratory Medicine,Faculty of Medicine,Division of Molecular Medicine and Gene Therapy(Swepub:lu)molm-kol (author)
- Zhang, Y (author)
- Flygare, JohanLund University,Lunds universitet,Avdelningen för molekylärmedicin och genterapi,Institutionen för laboratoriemedicin,Medicinska fakulteten,Division of Molecular Medicine and Gene Therapy,Department of Laboratory Medicine,Faculty of Medicine(Swepub:lu)molm-jfl (author)
- Lindström, M SKarolinska Institutet (author)
- Bryder, DavidLund University,Lunds universitet,Avdelningen för molekylär hematologi,Institutionen för laboratoriemedicin,Medicinska fakulteten,Division of Molecular Hematology (DMH),Department of Laboratory Medicine,Faculty of Medicine(Swepub:lu)med-dbr (author)
- Karlsson, StefanLund University,Lunds universitet,Avdelningen för molekylärmedicin och genterapi,Institutionen för laboratoriemedicin,Medicinska fakulteten,Division of Molecular Medicine and Gene Therapy,Department of Laboratory Medicine,Faculty of Medicine(Swepub:lu)molm-ska (author)
- Avdelningen för molekylär hematologiInstitutionen för laboratoriemedicin (creator_code:org_t)
Related titles
- In:Leukemia: Springer Science and Business Media LLC29:11, s. 2221-22291476-55510887-6924
Internet link
- https://portal.research.lu.se/files/1401027/8571889.pdfFULLTEXT
- http://www.ncbi.nlm.nih.gov/pubmed/25987256?dopt=AbstractFULLTEXT
- http://dx.doi.org/10.1038/leu.2015.128FULLTEXT
- https://www.nature.com/articles/leu2015128.pdf
- https://lup.lub.lu.se/record/5448846
- https://doi.org/10.1038/leu.2015.128
- http://kipublications.ki.se/Default.aspx?queryparsed=id:132393491
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