A mitochondria-specific isoform of FASTK is present in mitochondrial RNA granules and regulates gene expression and function.

  title={A mitochondria-specific isoform of FASTK is present in mitochondrial RNA granules and regulates gene expression and function.},
  author={Alexis A. Jourdain and Mirko Koppen and Chris D. M. Rodley and Kinsey Maundrell and Na{\"i}g Gueguen and Pascal Reynier and Adela M. Guaras and Jos{\'e} Antonio Enr{\'i}quez and Paul Anderson and Mar{\'i}a Simarro and Jean-Claude Martinou},
  journal={Cell reports},
  volume={10 7},

Role of FAST Kinase Domains 3 (FASTKD3) in Post-transcriptional Regulation of Mitochondrial Gene Expression*

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RNA Granules in the Mitochondria and Their Organization under Mitochondrial Stresses

Findings underline the important link between mitochondrial maintenance and the efficient expression of its genome and play an essential role in mitochondrial gene expression.

FASTKD2 is an RNA-binding protein required for mitochondrial RNA processing and translation.

Key aspects of the molecular network of a previously uncharacterized, disease-relevant RNA-binding protein, FASTKD2, are identified by a combination of genomic, molecular, and metabolic analyses.

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Transcription, Processing, and Decay of Mitochondrial RNA in Health and Disease

The essential steps of mitochondrial RNA synthesis, maturation, and degradation, the factors controlling these processes, and how the alteration of these processes is associated with human pathologies are described.

The mammalian mitochondrial epitranscriptome

Regulation of mitochondrial RNA expression by FASTK proteins

Structural modeling and mutagenesis studies suggested a similarity of a domain of the FASTK proteins to an endonuclease, and the N terminal region appears to resemble PPR proteins and determines if the protein is incorporated into mitochondrial RNA granules or not.

The FASTK family proteins fine-tune mitochondrial RNA processing

It is revealed that the FASTK protein family members are crucial regulators of non-canonical junction and non-coding mitochondrial RNA processing.

Is mitochondrial gene expression coordinated or stochastic?

Recently identified examples of the co-ordinated and stochastic processes that govern the mitochondrial transcriptome are reviewed to reveal the complexity of mitochondrial gene expression and the need for its in-depth exploration to understand how these organelles can respond to the energy demands of the cell.

Mitochondrial Gene Expression and Beyond—Novel Aspects of Cellular Physiology

The complex turnover of mitochondrial transcripts is summarized and an increasing body of evidence indicating new functions of mitochondria transcripts is presented, highlighting the importance of emerging aspects of mitochondrial gene regulation in human health and disease.



Alternative translation initiation augments the human mitochondrial proteome

DATI expands the human mitochondrial proteome by targeting a member of the cytosolic poly(A) binding protein family, PABPC5, and of the RNA/DNA helicase PIF1α, and most of the candidates identified by the screen are not currently annotated as mitochondrial proteins, and so dATI expanding the human mitochondome.

The human mitochondrial transcriptome and the RNA‐binding proteins that regulate its expression

This work has shown how changes in mitochondrial RNAs and their binding proteins can have significant effects on human health and opens new avenues for investigation of mitochondrial RNA‐binding proteins and the mechanisms by which they regulate mitochondrial gene expression.

A human mitochondrial poly(A) polymerase mutation reveals the complexities of post-transcriptional mitochondrial gene expression

It is concluded that the polymerase activity of mtPAP can be modulated by the presence of LRPPRC/SLIRP, and the alteration in poly(A) length is sufficient to cause dysregulation of post-transcriptional expression and the pathogenic lack of respiratory chain complexes.