A late-stage assembly checkpoint of the human mitochondrial ribosome large subunit
| العنوان: | A late-stage assembly checkpoint of the human mitochondrial ribosome large subunit |
|---|---|
| المؤلفون: | Leonor Miller-Fleming, Van Haute L, Rogan Jf, Adam M. Dinan, Andrew E. Firth, Alan J. Warren, Alexander J. Whitworth, Simone Pellegrino, Pedro Rebelo-Guiomar, Schraga Schwartz, Kyle Dent, Michal Minczuk, Sas Chen A, Caterina Garone, Byron Andrews |
| المساهمون: | Rebelo-Guiomar, Pedro [0000-0002-5060-7519], Pellegrino, Simone [0000-0001-6302-2774], Van Haute, Lindsey [0000-0001-7809-1473], Dinan, Adam [0000-0003-2812-1616], Firth, Andrew [0000-0002-7986-9520], Whitworth, Alex [0000-0002-1154-6629], Warren, Alan [0000-0001-9277-4553], Minczuk, Michal [0000-0001-8242-1420], Apollo - University of Cambridge Repository, Firth, Andrew E [0000-0002-7986-9520], Whitworth, Alexander J [0000-0002-1154-6629], Warren, Alan J [0000-0001-9277-4553], Rebelo-Guiomar, Pedro, Pellegrino, Simone, Dent, Kyle C, Sas-Chen, Aldema, Miller-Fleming, Leonor, Garone, Caterina, Van Haute, Lindsey, Rogan, Jack F, Dinan, Adam, Firth, Andrew E, Andrews, Byron, Whitworth, Alexander J, Schwartz, Schraga, Warren, Alan J, Minczuk, Michal |
| بيانات النشر: | Springer Science and Business Media LLC, 2022. |
| سنة النشر: | 2022 |
| مصطلحات موضوعية: | Male, Mitochondrial translation, 631/535/1258/1259, Ribosome biogenesis, General Physics and Astronomy, 13, Transcriptome, Mitochondrial Ribosomes, Gene Knockout Techniques, HEK293 Cell, RNA, Ribosomal, 16S, Mitochondrial ribosome, Drosophila Proteins, Methyltransferase, 64, Multidisciplinary, 631/45/500, Gene Knockout Technique, article, Mitochondrial Ribosome, Cell biology, 64/24, Drosophila melanogaster, Essential gene, 38/77, Human, Ribosomal Proteins, 101, Protein subunit, 101/58, 631/337/1645, Biology, Methylation, General Biochemistry, Genetics and Molecular Biology, Ribosomal Protein, 38, 38/91, Animals, Humans, Animal, 101/28, RNA, General Chemistry, Methyltransferases, HEK293 Cells, Protein Biosynthesis, Drosophila Protein, 631/337/574/1789, Ribosome Subunits, Large, 631/80/642/333, Biogenesis |
| الوصف: | Funder: Kay Kendall Leukaemia Fund (KKLF); doi: https://doi.org/10.13039/501100000402Test Funder: EC | EC Seventh Framework Programm | FP7 People: Marie-Curie Actions (FP7-PEOPLE - Specific Programme "People" Implementing the Seventh Framework Programme of the European Community for Research, Technological Development and Demonstration Activities (2007 to 2013)); doi: https://doi.org/10.13039/100011264Test; Grant(s): Mitobiopath-705560 Many cellular processes, including ribosome biogenesis, are regulated through post-transcriptional RNA modifications. Here, a genome-wide analysis of the human mitochondrial transcriptome shows that 2'-O-methylation is limited to residues of the mitoribosomal large subunit (mtLSU) 16S mt-rRNA, introduced by MRM1, MRM2 and MRM3, with the modifications installed by the latter two proteins being interdependent. MRM2 controls mitochondrial respiration by regulating mitoribosome biogenesis. In its absence, mtLSU particles (visualized by cryo-EM at the resolution of 2.6 ��) present disordered RNA domains, partial occupancy of bL36m and bound MALSU1:L0R8F8:mtACP anti-association module, allowing five mtLSU biogenesis intermediates with different intersubunit interface configurations to be placed along the assembly pathway. However, mitoribosome biogenesis does not depend on the methyltransferase activity of MRM2. Disruption of the MRM2 Drosophila melanogaster orthologue leads to mitochondria-related developmental arrest. This work identifies a key checkpoint during mtLSU assembly, essential to maintain mitochondrial homeostasis. |
| وصف الملف: | application/pdf; application/zip; text/xml; ELETTRONICO |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5f18440471c37b12da7c1a0d1c0ea4fbTest https://www.repository.cam.ac.uk/handle/1810/334157Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....5f18440471c37b12da7c1a0d1c0ea4fb |
| قاعدة البيانات: | OpenAIRE |
| الوصف غير متاح. |