دورية أكاديمية
PTBP1 promotes hematopoietic stem cell maintenance and red blood cell development by ensuring sufficient availability of ribosomal constituents.
| العنوان: | PTBP1 promotes hematopoietic stem cell maintenance and red blood cell development by ensuring sufficient availability of ribosomal constituents. |
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| المؤلفون: | Rehn, M., Wenzel, A., Frank, A.K., Schuster, M.B., Pundhir, S., Jørgensen, N., Vitting-Seerup, K., Ge, Y., Jendholm, J., Michaut, M., Schoof, E.M., Jensen, T.L., Rapin, N., Sapio, R.T., Andersen, K.L., Lund, A.H., Solimena, M., Holzenberger, M., Pestov, D.G., Porse, B.T. |
| المصدر: | Cell Rep. 39:110793 (2022) |
| سنة النشر: | 2022 |
| المجموعة: | PuSH - Publikationsserver des Helmholtz Zentrums München |
| مصطلحات موضوعية: | Cp: Molecular Biology, Cp: Stem Cell Research, Hematopoietic Stem Cells, Protein Synthesis, Ptbp1, Red Blood Cell Development, Ribosome Assembly |
| الوصف: | Ribosomopathies constitute a range of disorders associated with defective protein synthesis mainly affecting hematopoietic stem cells (HSCs) and erythroid development. Here, we demonstrate that deletion of poly-pyrimidine-tract-binding protein 1 (PTBP1) in the hematopoietic compartment leads to the development of a ribosomopathy-like condition. Specifically, loss of PTBP1 is associated with decreases in HSC self-renewal, erythroid differentiation, and protein synthesis. Consistent with its function as a splicing regulator, PTBP1 deficiency results in splicing defects in hundreds of genes, and we demonstrate that the up-regulation of a specific isoform of CDC42 partly mimics the protein-synthesis defect associated with loss of PTBP1. Furthermore, PTBP1 deficiency is associated with a marked defect in ribosome biogenesis and a selective reduction in the translation of mRNAs encoding ribosomal proteins. Collectively, this work identifies PTBP1 as a key integrator of ribosomal functions and highlights the broad functional repertoire of RNA-binding proteins. |
| نوع الوثيقة: | article in journal/newspaper |
| وصف الملف: | application/pdf |
| اللغة: | English |
| تدمد: | 2211-1247 |
| العلاقة: | info:eu-repo/semantics/altIdentifier/pmid/35545054; info:eu-repo/semantics/altIdentifier/wos/WOS:000798722200002; info:eu-repo/semantics/altIdentifier/isbn/2211-1247; info:eu-repo/semanti; https://push-zb.helmholtz-muenchen.de/frontdoor.php?source_opus=64980Test; urn:isbn:2211-1247; urn:issn:2211-1247 |
| DOI: | 10.1016/j.celrep.2022.110793 |
| الإتاحة: | https://doi.org/10.1016/j.celrep.2022.110793Test https://push-zb.helmholtz-muenchen.de/frontdoor.php?source_opus=64980Test |
| حقوق: | info:eu-repo/semantics/openAccess |
| رقم الانضمام: | edsbas.3C87E543 |
| قاعدة البيانات: | BASE |
Full Text Finder | تدمد: | 22111247 |
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| DOI: | 10.1016/j.celrep.2022.110793 |