Rackham 2016 Cell Rep

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Rackham O, Busch JD, Matic S, Siira SJ, Kuznetsova I, Atanassov I, Ermer JA, Shearwood AM, Richman TR, Stewart JB, Mourier A, Milenkovic D, Larsson NG, Filipovska A (2016) Hierarchical RNA processing is required for mitochondrial ribosome assembly. Cell Rep 16:1874-90.

» PMID: 27498866

Rackham O, Busch JD, Matic S, Siira SJ, Kuznetsova I, Atanassov I, Ermer JA, Shearwood AM, Richman TR, Stewart JB, Mourier A, Milenkovic D, Larsson NG, Filipovska A (2016) Cell Rep

Abstract: The regulation of mitochondrial RNA processing and its importance for ribosome biogenesis and energy metabolism are not clear. We generated conditional knockout mice of the endoribonuclease component of the RNase P complex, MRPP3, and report that it is essential for life and that heart and skeletal-muscle-specific knockout leads to severe cardiomyopathy, indicating that its activity is non-redundant. Transcriptome-wide parallel analyses of RNA ends (PARE) and RNA-seq enabled us to identify that in vivo 5' tRNA cleavage precedes 3' tRNA processing, and this is required for the correct biogenesis of the mitochondrial ribosomal subunits. We identify that mitoribosomal biogenesis proceeds co-transcriptionally because large mitoribosomal proteins can form a subcomplex on an unprocessed RNA containing the 16S rRNA. Taken together, our data show that RNA processing links transcription to translation via assembly of the mitoribosome.

Copyright © 2016 The Author(s). Published by Elsevier Inc. All rights reserved.

Keywords: PPR domains, RNA metabolism, RNA-seq, Mitochondria

O2k-Network Lab: DE Cologne Larsson NG, FR Bordeaux Devin A, AU Perth Filipovska A


Labels: MiParea: Respiration, mt-Biogenesis;mt-density, Genetic knockout;overexpression 


Organism: Mouse  Tissue;cell: Heart  Preparation: Isolated mitochondria  Enzyme: Complex I, Complex II;succinate dehydrogenase, Complex III, Complex IV;cytochrome c oxidase, Complex V;ATP synthase 

Coupling state: LEAK, OXPHOS, ET  Pathway: N, S, CIV, NS, Other combinations  HRR: Oxygraph-2k 

2016-10