Mitochondrial transcription and translation: overview

Mitochondrial DNA replication in mammalian cells: overview of the pathway

(2018) Maria Falkenberg   Essays in Biochemistry

Mitochondrial DNA transcription and translation: clinical syndromes

(2018) Veronika Boczonadi et al.   Essays in Biochemistry

Metabolic and chemical regulation of tRNA modification associated with taurine deficiency and human disease

(2018) Kana Asano et al.   NUCLEIC ACIDS RESEARCH

Polyadenylation and degradation of structurally abnormal mitochondrial tRNAs in human cells

(2018) Marina Toompuu et al.   NUCLEIC ACIDS RESEARCH

Mitochondrial Genome Engineering: The Revolution May Not Be CRISPR-Ized

(2018) Payam A. Gammage et al.   TRENDS IN GENETICS

The Pseudouridine Synthase RPUSD4 Is an Essential Component of Mitochondrial RNA Granules

(2017) Sofia Zaganelli et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

FASTKD1 and FASTKD4 have opposite effects on expression of specific mitochondrial RNAs, depending upon their endonuclease-like RAP domain

(2017) Erik Boehm et al.   NUCLEIC ACIDS RESEARCH

Regulation of Mammalian Mitochondrial Gene Expression: Recent Advances

(2017) Sarah F. Pearce et al.   TRENDS IN BIOCHEMICAL SCIENCES

LRPPRC-mediated folding of the mitochondrial transcriptome

(2017) Stefan J. Siira et al.   Nature Communications

Maturation of selected human mitochondrial tRNAs requires deadenylation

(2017) Sarah F Pearce et al.   eLife

Mitochondrial Protein Synthesis Adapts to Influx of Nuclear-Encoded Protein

(2016) Ricarda Richter-Dennerlein et al.   CELL

A Genome-wide CRISPR Death Screen Identifies Genes Essential for Oxidative Phosphorylation

(2016) Jason D. Arroyo et al.   Cell Metabolism

NSUN3 and ABH1 modify the wobble position of mt‐tRNAMetto expand codon recognition in mitochondrial translation

(2016) Sara Haag et al.   EMBO JOURNAL

A pseudouridine synthase module is essential for mitochondrial protein synthesis and cell viability

(2016) Hana Antonicka et al.   EMBO REPORTS

Human Mitochondrial Transcription Factor B2 Is Required for Promoter Melting during Initiation of Transcription

(2016) Viktor Posse et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

NSUN3 methylase initiates 5-formylcytidine biogenesis in human mitochondrial tRNAMet

(2016) Saori Nakano et al.   Nature Chemical Biology

Mitochondrial transcription termination factor 1 directs polar replication fork pausing

(2016) Yonghong Shi et al.   NUCLEIC ACIDS RESEARCH

Human mitochondrial transcription factors TFAM and TFB2M work synergistically in promoter melting during transcription initiation

(2016) Aparna Ramachandran et al.   NUCLEIC ACIDS RESEARCH

Human mitochondrial ribosomes can switch their structural RNA composition

(2016) Joanna Rorbach et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Deficient methylation and formylation of mt-tRNAMet wobble cytosine in a patient carrying mutations in NSUN3

(2016) Lindsey Van Haute et al.   Nature Communications

Mitochondrial 16S rRNA Is Methylated by tRNA Methyltransferase TRMT61B in All Vertebrates

(2016) Dan Bar-Yaacov et al.   PLOS BIOLOGY

Mtu1-Mediated Thiouridine Formation of Mitochondrial tRNAs Is Required for Mitochondrial Translation and Is Involved in Reversible Infantile Liver Injury

(2016) Yong Wu et al.   PLoS Genetics

Human Cells Require Non-stop Ribosome Rescue Activity in Mitochondria

(2016) Heather A. Feaga et al.   PLoS Genetics

TRMT5 Mutations Cause a Defect in Post-transcriptional Modification of Mitochondrial tRNA Associated with Multiple Respiratory-Chain Deficiencies

(2015) Christopher A. Powell et al.   AMERICAN JOURNAL OF HUMAN GENETICS

RMND1 deficiency associated with neonatal lactic acidosis, infantile onset renal failure, deafness and multiorgan involvement

(2015) Alexandre Janer et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

TEFM is a potent stimulator of mitochondrial transcription elongation in vitro

(2015) Viktor Posse et al.   NUCLEIC ACIDS RESEARCH

Structure of mitochondrial poly(A) RNA polymerase reveals the structural basis for dimerization, ATP selectivity and the SPAX4 disease phenotype

(2015) Mikalai Lapkouski et al.   NUCLEIC ACIDS RESEARCH

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

(2015) Johannes Popow et al.   RNA

Replication-transcription switch in human mitochondria

(2015) K. Agaronyan et al.   SCIENCE

The complete structure of the 55S mammalian mitochondrial ribosome

(2015) B. J. Greber et al.   SCIENCE

The structure of the human mitochondrial ribosome

(2015) A. Amunts et al.   SCIENCE

A Mitochondria-Specific Isoform of FASTK Is Present In Mitochondrial RNA Granules and Regulates Gene Expression and Function

(2015) Alexis A. Jourdain et al.   Cell Reports

Mitochondrial RNA Granules Are Centers for Posttranscriptional RNA Processing and Ribosome Biogenesis

(2015) Hana Antonicka et al.   Cell Reports

SLIRP Regulates the Rate of Mitochondrial Protein Synthesis and Protects LRPPRC from Degradation

(2015) Marie Lagouge et al.   PLoS Genetics

Response to “Ribosome Rescue and Translation Termination at Non-standard Stop Codons by ICT1 in Mammalian Mitochondria”

(2015) Zofia Maria Chrzanowska-Lightowlers et al.   PLoS Genetics

Glutamyl-tRNAGlnamidotransferase is essential for mammalian mitochondrial translationin vivo

(2014) Lucía Echevarría et al.   BIOCHEMICAL JOURNAL

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

(2014) William C. Wilson et al.   HUMAN MOLECULAR GENETICS

Loss of LRPPRC causes ATP synthase deficiency

(2014) A. Mourier et al.   HUMAN MOLECULAR GENETICS

Assignment of 2′-O-Methyltransferases to Modification Sites on the Mammalian Mitochondrial Large Subunit 16 S Ribosomal RNA (rRNA)

(2014) Ken-Wing Lee et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

MRM2 and MRM3 are involved in biogenesis of the large subunit of the mitochondrial ribosome

(2014) Joanna Rorbach et al.   MOLECULAR BIOLOGY OF THE CELL

The complete structure of the large subunit of the mammalian mitochondrial ribosome

(2014) Basil J. Greber et al.   NATURE

Cryo-EM structure of the small subunit of the mammalian mitochondrial ribosome

(2014) P. S. Kaushal et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Structure of the large ribosomal subunit from human mitochondria

(2014) A. Brown et al.   SCIENCE

Ribosome Rescue and Translation Termination at Non-Standard Stop Codons by ICT1 in Mammalian Mitochondria

(2014) Shiori Akabane et al.   PLoS Genetics

Defective i6A37 Modification of Mitochondrial and Cytosolic tRNAs Results from Pathogenic Mutations in TRIT1 and Its Substrate tRNA

(2014) John W. Yarham et al.   PLoS Genetics

NSUN4 Is a Dual Function Mitochondrial Protein Required for Both Methylation of 12S rRNA and Coordination of Mitoribosomal Assembly

(2014) Metodi Dimitrov Metodiev et al.   PLoS Genetics

GRSF1 Regulates RNA Processing in Mitochondrial RNA Granules

(2013) Alexis A. Jourdain et al.   Cell Metabolism

MTERF1 Binds mtDNA to Prevent Transcriptional Interference at the Light-Strand Promoter but Is Dispensable for rRNA Gene Transcription Regulation

(2013) Mügen Terzioglu et al.   Cell Metabolism

The Mitochondrial RNA-Binding Protein GRSF1 Localizes to RNA Granules and Is Required for Posttranscriptional Mitochondrial Gene Expression

(2013) Hana Antonicka et al.   Cell Metabolism

Structural basis of reverse nucleotide polymerization

(2013) A. Nakamura et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Codon-reading specificities of mitochondrial release factors and translation termination at non-standard stop codons

(2013) Christoffer Lind et al.   Nature Communications

Mutations in C12orf62, a Factor that Couples COX I Synthesis with Cytochrome c Oxidase Assembly, Cause Fatal Neonatal Lactic Acidosis

(2012) Woranontee Weraarpachai et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Structure based hypothesis of a mitochondrial ribosome rescue mechanism

(2012) Martijn A Huynen et al.   Biology Direct

LRPPRC/SLIRP suppresses PNPase-mediated mRNA decay and promotes polyadenylation in human mitochondria

(2012) Takeshi Chujo et al.   NUCLEIC ACIDS RESEARCH

Human mitochondrial RNA decay mediated by PNPase–hSuv3 complex takes place in distinct foci

(2012) Lukasz S. Borowski et al.   NUCLEIC ACIDS RESEARCH

Trmt61B is a methyltransferase responsible for 1-methyladenosine at position 58 of human mitochondrial tRNAs

(2012) T. Chujo et al.   RNA

A Combinatorial Amino Acid Code for RNA Recognition by Pentatricopeptide Repeat Proteins

(2012) Alice Barkan et al.   PLoS Genetics

Iterative orthology prediction uncovers new mitochondrial proteins and identifies C12orf62 as the human ortholog of COX14, a protein involved in the assembly of cytochrome c oxidase

(2012) Radek Szklarczyk et al.   GENOME BIOLOGY

RNA processing in human mitochondria

(2011) Maria I.G. Lopez Sanchez et al.   CELL CYCLE

LRPPRC is necessary for polyadenylation and coordination of translation of mitochondrial mRNAs

(2011) Benedetta Ruzzenente et al.   EMBO JOURNAL

The 2-thiouridylase function of the human MTU1 (TRMU) enzyme is dispensable for mitochondrial translation

(2011) F. Sasarman et al.   HUMAN MOLECULAR GENETICS

Termination of Protein Synthesis in Mammalian Mitochondria

(2011) Zofia M. A. Chrzanowska-Lightowlers et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Structural Basis for Dimerization and Activity of Human PAPD1, a Noncanonical Poly(A) Polymerase

(2011) Yun Bai et al.   MOLECULAR CELL

Structure of human mitochondrial RNA polymerase

(2011) Rieke Ringel et al.   NATURE

TEFM (c17orf42) is necessary for transcription of human mtDNA

(2011) Michal Minczuk et al.   NUCLEIC ACIDS RESEARCH

PDE12 removes mitochondrial RNA poly(A) tails and controls translation in human mitochondria

(2011) Joanna Rorbach et al.   NUCLEIC ACIDS RESEARCH

Localization of Human RNase Z Isoforms: Dual Nuclear/Mitochondrial Targeting of the ELAC2 Gene Product by Alternative Translation Initiation

(2011) Walter Rossmanith   PLoS One

Involvement of human ELAC2 gene product in 3' end processing of mitochondrial tRNAs

(2011) Lien K. Brzezniak et al.   RNA Biology

LRPPRC is a mitochondrial matrix protein that is conserved in metazoans

(2010) Fredrik H. Sterky et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Human ERAL1 is a mitochondrial RNA chaperone involved in the assembly of the 28S small mitochondrial ribosomal subunit

(2010) Sven Dennerlein et al.   BIOCHEMICAL JOURNAL

Human mitochondrial mRNAs—like members of all families, similar but different

(2010) Richard J. Temperley et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Effects on mitochondrial transcription of manipulating mTERF protein levels in cultured human HEK293 cells

(2010) Anne K Hyvärinen et al.   BMC MOLECULAR BIOLOGY

Helix Unwinding and Base Flipping Enable Human MTERF1 to Terminate Mitochondrial Transcription

(2010) Elena Yakubovskaya et al.   CELL

A functional peptidyl-tRNA hydrolase, ICT1, has been recruited into the human mitochondrial ribosome

(2010) Ricarda Richter et al.   EMBO JOURNAL

Mitochondrial and Nuclear Genomic Responses to Loss ofLRPPRCExpression

(2010) Vishal M. Gohil et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Human Mitochondrial Transcription Revisited

(2010) Dmitry Litonin et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

LRPPRC and SLIRP Interact in a Ribonucleoprotein Complex That Regulates Posttranscriptional Gene Expression in Mitochondria

(2010) Florin Sasarman et al.   MOLECULAR BIOLOGY OF THE CELL

Human mitochondrial mTERF wraps around DNA through a left-handed superhelical tandem repeat

(2010) Nereida Jiménez-Menéndez et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Targeting of the cytosolic poly(A) binding protein PABPC1 to mitochondria causes mitochondrial translation inhibition

(2010) Mateusz Wydro et al.   NUCLEIC ACIDS RESEARCH

G-quadruplex structures in RNA stimulate mitochondrial transcription termination and primer formation

(2010) P. H. Wanrooij et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Hungry Codons Promote Frameshifting in Human Mitochondrial Ribosomes

(2010) R. Temperley et al.   SCIENCE

TFB2 Is a Transient Component of the Catalytic Site of the Human Mitochondrial RNA Polymerase

(2009) Marina Sologub et al.   CELL

Methylation of 12S rRNA Is Necessary for In Vivo Stability of the Small Subunit of the Mammalian Mitochondrial Ribosome

(2009) Metodi D. Metodiev et al.   Cell Metabolism

EF-G2mt Is an Exclusive Recycling Factor in Mammalian Mitochondrial Protein Synthesis

(2009) Masafumi Tsuboi et al.   MOLECULAR CELL

Human mitochondrial RNA turnover caught in flagranti: involvement of hSuv3p helicase in RNA surveillance

(2009) Roman J. Szczesny et al.   NUCLEIC ACIDS RESEARCH

Biogenesis of glutaminyl-mt tRNAGln in human mitochondria

(2009) A. Nagao et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A Computational Screen for Regulators of Oxidative Phosphorylation Implicates SLIRP in Mitochondrial RNA Homeostasis

(2009) Joshua M. Baughman et al.   PLoS Genetics

RNase P without RNA: Identification and Functional Reconstitution of the Human Mitochondrial tRNA Processing Enzyme

(2008) Johann Holzmann et al.   CELL

Roles of the N- and C-Terminal Domains of Mammalian Mitochondrial Initiation Factor 3 in Protein Biosynthesis

(2008) Md. Emdadul Haque et al.   JOURNAL OF MOLECULAR BIOLOGY

Characterization of Human GTPBP3, a GTP-Binding Protein Involved in Mitochondrial tRNA Modification

(2008) M. Villarroya et al.   MOLECULAR AND CELLULAR BIOLOGY

A Single Mammalian Mitochondrial Translation Initiation Factor Functionally Replaces Two Bacterial Factors

(2008) Rahul Gaur et al.   MOLECULAR CELL

The human mitochondrial ribosome recycling factor is essential for cell viability

(2008) Joanna Rorbach et al.   NUCLEIC ACIDS RESEARCH