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

Mitochondrial miRNA determines chemoresistance by reprogramming metabolism and regulating mitochondrial transcription

(2019) Song Fan et al.   CANCER RESEARCH

HIF-1α protects against oxidative stress by directly targeting mitochondria

(2019) Hong-Sheng Li et al.   Redox Biology

Mitochondrial RNA granules are critically dependent on mtDNA replication factors Twinkle and mtSSB

(2019) Fenna Hensen et al.   NUCLEIC ACIDS RESEARCH

NLRX1 resides in mitochondrial RNA granules and regulates mitochondrial RNA processing and bioenergetic adaptation

(2018) Kritarth Singh et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Mitochondrial DNA transcription and translation: clinical syndromes

(2018) Veronika Boczonadi et al.   Essays in Biochemistry

The mitochondrial transcription factor TFAM in neurodegeneration: emerging evidence and mechanisms

(2018) Inhae Kang et al.   FEBS LETTERS

A common pattern of DNase I footprinting throughout the human mtDNA unveils clues for a chromatin-like organization

(2018) Amit Blumberg et al.   GENOME RESEARCH

Mitochondria Transcription Factor A: A Putative Target for the Effect of Melatonin on U87MG Malignant Glioma Cell Line

(2018) Daiane Franco et al.   MOLECULES

Eukaryotic RNA 5′-End NAD + Capping and DeNADding

(2018) Megerditch Kiledjian   TRENDS IN CELL BIOLOGY

Concerted regulation of mitochondrial and nuclear non‐coding RNAs by a dual‐targeted RNase Z

(2018) Stefan J Siira et al.   EMBO REPORTS

Stress and glucocorticoid receptor regulation of mitochondrial gene expression

(2018) Hannah E. Lapp et al.   JOURNAL OF MOLECULAR ENDOCRINOLOGY

Mitochondrial double-stranded RNA triggers antiviral signalling in humans

(2018) Ashish Dhir et al.   NATURE

The mammalian mitochondrial epitranscriptome

(2018) Pedro Rebelo-Guiomar et al.   Biochimica et Biophysica Acta-Gene Regulatory Mechanisms

mTOR as a central hub of nutrient signalling and cell growth

(2018) Joungmok Kim et al.   NATURE CELL BIOLOGY

Polynucleotide phosphorylase: Not merely an RNase but a pivotal post-transcriptional regulator

(2018) Todd A. Cameron et al.   PLoS Genetics

Exploring the mitochondrial microRNA import pathway through Polynucleotide Phosphorylase (PNPase)

(2017) Danielle L. Shepherd et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

Identification of FASTKD2 compound heterozygous mutations as the underlying cause of autosomal recessive MELAS-like syndrome

(2017) Da Hye Yoo et al.   MITOCHONDRION

The FASTK family of proteins: emerging regulators of mitochondrial RNA biology

(2017) Alexis A. Jourdain et al.   NUCLEIC ACIDS RESEARCH

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

The human mitochondrial transcription factor A is a versatile G-quadruplex binding protein

(2017) Sébastien Lyonnais et al.   Scientific Reports

Unexpected sequences and structures of mtDNA required for efficient transcription from the first heavy-strand promoter

(2017) Akira Uchida et al.   eLife

Recessive Mutations in TRMT10C Cause Defects in Mitochondrial RNA Processing and Multiple Respiratory Chain Deficiencies

(2016) Metodi D. Metodiev et al.   AMERICAN JOURNAL OF HUMAN GENETICS

MOF Acetyl Transferase Regulates Transcription and Respiration in Mitochondria

(2016) Aindrila Chatterjee et al.   CELL

The process of mammalian mitochondrial protein synthesis

(2016) Nicole Mai et al.   CELL AND TISSUE RESEARCH

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

(2016) Erik Boehm et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mutations in TFAM, encoding mitochondrial transcription factor A, cause neonatal liver failure associated with mtDNA depletion

(2016) Ashlee R. Stiles et al.   MOLECULAR GENETICS AND METABOLISM

p53 coordinates base excision repair to prevent genomic instability

(2016) Mattia Poletto et al.   NUCLEIC ACIDS RESEARCH

A novelHSD17B10mutation impairing the activities of the mitochondrial RNase P complex causes X-linked intractable epilepsy and neurodevelopmental regression

(2016) Marni J. Falk et al.   RNA Biology

Osteogenic differentiation of human MSCs: Specific occupancy of the mitochondrial DNA by NFATc1 transcription factor

(2015) Elisabetta Lambertini et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Mitochondrial transcript maturation and its disorders

(2015) Lindsey Van Haute et al.   JOURNAL OF INHERITED METABOLIC DISEASE

A model for transcription initiation in human mitochondria

(2015) Yaroslav I. Morozov et al.   NUCLEIC ACIDS RESEARCH

TEFM is a potent stimulator of mitochondrial transcription elongation in vitro

(2015) Viktor Posse 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 structure of the human mitochondrial ribosome

(2015) A. Amunts et al.   SCIENCE

Mitochondriolus: assembling mitoribosomes

(2015) Antoni Barrientos   Oncotarget

The Human Mitochondrial DEAD-Box Protein DDX28 Resides in RNA Granules and Functions in Mitoribosome Assembly

(2015) Ya-Ting Tu et al.   Cell Reports

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

A Complex Genome-MicroRNA Interplay in Human Mitochondria

(2015) Santosh Shinde et al.   Biomed Research International

In D-loop: 40years of mitochondrial 7S DNA

(2014) Thomas J. Nicholls et al.   EXPERIMENTAL GERONTOLOGY

Tissue-specific responses to the LRPPRC founder mutation in French Canadian Leigh Syndrome

(2014) F. Sasarman et al.   HUMAN MOLECULAR GENETICS

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

Stat3 Binds to mtDNA and Regulates Mitochondrial Gene Expression in Keratinocytes

(2014) Everardo Macias et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

NAD captureSeq indicates NAD as a bacterial cap for a subset of regulatory RNAs

(2014) Hana Cahová et al.   NATURE

Evidence for Site-Specific Occupancy of the Mitochondrial Genome by Nuclear Transcription Factors

(2014) Georgi K. Marinov et al.   PLoS One

Autophagy Inhibitor LRPPRC Suppresses Mitophagy through Interaction with Mitophagy Initiator Parkin

(2014) Jing Zou et al.   PLoS One

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

Functional Genomic Analysis of Human Mitochondrial RNA Processing

(2014) Ashley R. Wolf et al.   Cell Reports

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

MitomiRs delineating the intracellular localization of microRNAs at mitochondria

(2013) S. Bandiera et al.   FREE RADICAL BIOLOGY AND MEDICINE

Mitochondrial Ribosomal RNA (rRNA) Methyltransferase Family Members Are Positioned to Modify Nascent rRNA in Foci near the Mitochondrial DNA Nucleoid

(2013) Ken-Wing Lee et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

REXO2 Is an Oligoribonuclease Active in Human Mitochondria

(2013) Francesco Bruni et al.   PLoS One

A Systems Approach for Decoding Mitochondrial Retrograde Signaling Pathways

(2013) S. Chae et al.   Science Signaling

Mutation in PNPT1 , which Encodes a Polyribonucleotide Nucleotidyltransferase, Impairs RNA Import into Mitochondria and Causes Respiratory-Chain Deficiency

(2012) Vanessa Vedrenne et al.   AMERICAN JOURNAL OF HUMAN GENETICS

A Mutation in PNPT1 , Encoding Mitochondrial-RNA-Import Protein PNPase, Causes Hereditary Hearing Loss

(2012) Simon von Ameln et al.   AMERICAN JOURNAL OF HUMAN GENETICS

The post-transcriptional life of mammalian mitochondrial RNA

(2012) Joanna Rorbach et al.   BIOCHEMICAL JOURNAL

p53 Opens the Mitochondrial Permeability Transition Pore to Trigger Necrosis

(2012) Angelina V. Vaseva et al.   CELL

Role of leucine-rich pentatricopeptide repeat motif-containing protein (LRPPRC) for anti-apoptosis and tumourigenesis in cancers

(2012) Tian Tian et al.   EUROPEAN JOURNAL OF CANCER

Alternative translation initiation augments the human mitochondrial proteome

(2012) Lawrence Kazak et al.   NUCLEIC ACIDS RESEARCH

A subcomplex of human mitochondrial RNase P is a bifunctional methyltransferase—extensive moonlighting in mitochondrial tRNA biogenesis

(2012) Elisa Vilardo et al.   NUCLEIC ACIDS RESEARCH

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

Mammalian transcription factor A is a core component of the mitochondrial transcription machinery

(2012) Y. Shi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Transcriptional integration of mitochondrial biogenesis

(2012) Richard C. Scarpulla et al.   TRENDS IN ENDOCRINOLOGY AND METABOLISM

p53-Dependent Regulation of Mitochondrial Energy Production by the RelA Subunit of NF- B

(2011) R. F. Johnson et al.   CANCER RESEARCH

Processing mitochondrial (t)RNAs: New enzyme, old job

(2011) Walter Rossmanith et al.   CELL CYCLE

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

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

(2011) Yun Bai et al.   MOLECULAR CELL

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

Human 2′-phosphodiesterase localizes to the mitochondrial matrix with a putative function in mitochondrial RNA turnover

(2011) Jesper Buchhave Poulsen et al.   NUCLEIC ACIDS RESEARCH

Pre-microRNA and Mature microRNA in Human Mitochondria

(2011) Eric Barrey et al.   PLoS One

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

(2011) Walter Rossmanith   PLoS One

Nuclear Outsourcing of RNA Interference Components to Human Mitochondria

(2011) Simonetta Bandiera et al.   PLoS One

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

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

Defective Mitochondrial mRNA Maturation Is Associated with Spastic Ataxia

(2010) Andrew H. Crosby et al.   AMERICAN JOURNAL OF HUMAN GENETICS

LRPPRC is a mitochondrial matrix protein that is conserved in metazoans

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

Leucine-Rich Pentatricopeptide-Repeat Containing Protein Regulates Mitochondrial Transcription

(2010) Neal Sondheimer et al.   BIOCHEMISTRY

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

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

DNA condensation in bacteria: Interplay between macromolecular crowding and nucleoid proteins

(2010) Renko de Vries   BIOCHIMIE

Mitochondrial and Nuclear Genomic Responses to Loss ofLRPPRCExpression

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

Peroxisome Proliferator-activated Receptor γ Co-activator 1α (PGC-1α) and Sirtuin 1 (SIRT1) Reside in Mitochondria

(2010) Katia Aquilano 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

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

Loss of p53 causes mitochondrial DNA depletion and altered mitochondrial reactive oxygen species homeostasis

(2009) Maria A. Lebedeva et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Human Mitochondrial SUV3 and Polynucleotide Phosphorylase Form a 330-kDa Heteropentamer to Cooperatively Degrade Double-stranded RNA with a 3′-to-5′ Directionality

(2009) Dennis Ding-Hwa Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Pentatricopeptide repeat domain protein 1 lowers the levels of mitochondrial leucine tRNAs in cells

(2009) Oliver Rackham et al.   NUCLEIC ACIDS RESEARCH

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

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

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

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

Transcriptional co-expression and co-regulation of genes coding for components of the oxidative phosphorylation system

(2008) Corina van Waveren et al.   BMC GENOMICS

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

(2008) Johann Holzmann et al.   CELL

Steroid and thyroid hormone receptors in mitochondria

(2008) Anna-Maria G. Psarra et al.   IUBMB LIFE