Perks 2018 Cell Rep
| [[Has title::Perks KL, Rossetti G, Kuznetsova I, Hughes LA, Ermer JA, Ferreira N, Busch JD, Rudler DL, Spahr H, Schöndorf T, Shearwood AJ, Viola HM, Siira SJ, Hool LC, Milenkovic D, Larsson NG, Rackham O, Filipovska A (2018) PTCD1 is required for 16S rRNA maturation complex stability and mitochondrial ribosome assembly. Cell Rep 23:127-42.]] |
» [[Has info::PMID: 29617655 Open Access]]
Was written by::Perks KL, Was written by::Rossetti G, Was written by::Kuznetsova I, Was written by::Hughes LA, Was written by::Ermer JA, Was written by::Ferreira N, Was written by::Busch JD, Was written by::Rudler DL, Was written by::Spahr H, Was written by::Schoendorf T, Was written by::Shearwood AJ, Was written by::Viola HM, Was written by::Siira SJ, Was written by::Hool LC, Was written by::Milenkovic D, Was written by::Larsson NG, Was written by::Rackham O, Was written by::Filipovska A (Was published in year::2018) Was published in journal::Cell Rep
Abstract: [[has abstract::The regulation of mitochondrial RNA life cycles and their roles in ribosome biogenesis and energy metabolism are not fully understood. We used CRISPR/Cas9 to generate heart- and skeletal-muscle-specific knockout mice of the pentatricopeptide repeat domain protein 1, PTCD1, and show that its loss leads to severe cardiomyopathy and premature death. Our detailed transcriptome-wide and functional analyses of these mice enabled us to identify the molecular role of PTCD1 as a 16S rRNA-binding protein essential for its stability, pseudouridylation, and correct biogenesis of the mitochondrial large ribosomal subunit. We show that impaired mitoribosome biogenesis can have retrograde signaling effects on nuclear gene expression through the transcriptional activation of the mTOR pathway and upregulation of cytoplasmic protein synthesis and pro-survival factors in the absence of mitochondrial translation. Taken together, our data show that impaired assembly of the mitoribosome exerts its consequences via differential regulation of mitochondrial and cytoplasmic protein synthesis.]] • Keywords: has publicationkeywords::RNA, has publicationkeywords::RNA-binding proteins, has publicationkeywords::RNA-seq, has publicationkeywords::cardiomyopathy, has publicationkeywords::Mitochondria, has publicationkeywords::Mitochondrial gene expression, has publicationkeywords::Mitochondrial ribosome, has publicationkeywords::Regulatory RNAs, has publicationkeywords::Ribosome biogenesis • Bioblast editor: [[has editor::Kandolf G]] • O2k-Network Lab: Was published by MiPNetLab::AU Perth Filipovska A, Was published by MiPNetLab::DE Cologne Larsson NG
Labels: MiParea: MiP area::Respiration, MiP area::mt-Biogenesis;mt-density, MiP area::Genetic knockout;overexpression
Organism: Organism::Mouse
Tissue;cell: tissue and cell::Heart
Preparation: Preparation::Isolated mitochondria
Enzyme: Enzyme::Complex I, Enzyme::Complex II;succinate dehydrogenase, Enzyme::Complex III, Enzyme::Complex IV;cytochrome c oxidase, Enzyme::Complex V;ATP synthase
Coupling state: Coupling states::LEAK, Coupling states::OXPHOS, Coupling states::ET Pathway: Pathways::N, Pathways::Gp, Pathways::NS HRR: Instrument and method::Oxygraph-2k
