Anne-Marie J. Shearwood
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View article: Cardiolipin is required for membrane docking of mitochondrial ribosomes and protein synthesis
Cardiolipin is required for membrane docking of mitochondrial ribosomes and protein synthesis Open
The mitochondrial inner membrane contains a unique phospholipid known as cardiolipin (CL), which stabilises the protein complexes embedded in the membrane and supports its overall structure. Recent evidence indicates that the mitochondrial…
View article: Fidelity of translation initiation is required for coordinated respiratory complex assembly
Fidelity of translation initiation is required for coordinated respiratory complex assembly Open
The initiation of mitochondrial protein synthesis fine-tunes the assembly of respiratory complexes and energy production.
View article: Stress signaling and cellular proliferation reverse the effects of mitochondrial mistranslation
Stress signaling and cellular proliferation reverse the effects of mitochondrial mistranslation Open
View article: Misregulation of Mitochondrial Protein Synthesis Leads to Cardiomyopathy
Misregulation of Mitochondrial Protein Synthesis Leads to Cardiomyopathy Open
View article: Early-onset Parkinson disease caused by a mutation in CHCHD2 and mitochondrial dysfunction
Early-onset Parkinson disease caused by a mutation in CHCHD2 and mitochondrial dysfunction Open
Our findings implicate CHCHD2 in the pathogenesis of recessive early-onset PD, expanding the repertoire of mitochondrial proteins that play a direct role in this disease.
View article: Concerted regulation of mitochondrial and nuclear non‐coding <scp>RNA</scp> s by a dual‐targeted <scp>RN</scp> ase Z
Concerted regulation of mitochondrial and nuclear non‐coding <span>RNA</span> s by a dual‐targeted <span>RN</span> ase Z Open
View article: Adult-onset obesity is triggered by impaired mitochondrial gene expression
Adult-onset obesity is triggered by impaired mitochondrial gene expression Open
Reduction in an RNA binding protein impairs mitochondrial biogenesis and alters cell signaling, resulting in obesity.
View article: Simultaneous processing and degradation of mitochondrial RNAs revealed by circularized RNA sequencing
Simultaneous processing and degradation of mitochondrial RNAs revealed by circularized RNA sequencing Open
Mammalian mitochondrial RNAs are unique as they are derived from primary transcripts that encompass almost the entire mitochondrial genome. This necessitates extensive processing to release the individual mRNAs, rRNAs and tRNAs required fo…
View article: Transcriptome-wide effects of a<i>POLR3A</i>gene mutation in patients with an unusual phenotype of striatal involvement
Transcriptome-wide effects of a<i>POLR3A</i>gene mutation in patients with an unusual phenotype of striatal involvement Open
RNA polymerase III is essential for the transcription of non-coding RNAs, including tRNAs. Mutations in the genes encoding its largest subunits are known to cause hypomyelinating leukodystrophies (HLD7) with pathogenetic mechanisms hypothe…
View article: Hierarchical RNA Processing Is Required for Mitochondrial Ribosome Assembly
Hierarchical RNA Processing Is Required for Mitochondrial Ribosome Assembly Open
View article: Mutation in MRPS34 Compromises Protein Synthesis and Causes Mitochondrial Dysfunction
Mutation in MRPS34 Compromises Protein Synthesis and Causes Mitochondrial Dysfunction Open
The evolutionary divergence of mitochondrial ribosomes from their bacterial and cytoplasmic ancestors has resulted in reduced RNA content and the acquisition of mitochondria-specific proteins. The mitochondrial ribosomal protein of the sma…