TWINKLE is an essential mitochondrial helicase required for synthesis of nascent D-loop strands and complete mtDNA replication

Mitochondria: In Sickness and in Health

(2012) Jodi Nunnari et al.   CELL

Modeling Pathogenic Mutations of Human Twinkle in Drosophila Suggests an Apoptosis Role in Response to Mitochondrial Defects

(2012) Alvaro Sanchez-Martinez et al.   PLoS One

MTERF4 Regulates Translation by Targeting the Methyltransferase NSUN4 to the Mammalian Mitochondrial Ribosome

(2011) Yolanda Cámara et al.   Cell Metabolism

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

(2011) Benedetta Ruzzenente et al.   EMBO JOURNAL

Mitochondrial DNA mutations in disease and aging

(2011) Chan Bae Park et al.   JOURNAL OF CELL BIOLOGY

The mitochondrial transcription and packaging factor Tfam imposes a U-turn on mitochondrial DNA

(2011) Huu B Ngo et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

The mitochondrial DNA helicase TWINKLE can assemble on a closed circular template and support initiation of DNA synthesis

(2011) Elisabeth Jemt et al.   NUCLEIC ACIDS RESEARCH

Super-resolution microscopy reveals that mammalian mitochondrial nucleoids have a uniform size and frequently contain a single copy of mtDNA

(2011) C. Kukat et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Bicoid Stability Factor Controls Polyadenylation and Expression of Specific Mitochondrial mRNAs in Drosophila melanogaster

(2011) Ana Bratic et al.   PLoS Genetics

Somatic Mitochondrial DNA Mutations in Mammalian Aging

(2010) Nils-Göran Larsson   Annual Review of Biochemistry

The human mitochondrial replication fork in health and disease

(2010) Sjoerd Wanrooij et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

High mitochondrial DNA copy number has detrimental effects in mice

(2010) Emil Ylikallio et al.   HUMAN MOLECULAR GENETICS

Mammalian Mitochondrial DNA Replication Intermediates Are Essentially Duplex but Contain Extensive Tracts of RNA/DNA Hybrid

(2010) Jaakko L.O. Pohjoismäki et al.   JOURNAL OF MOLECULAR BIOLOGY

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

Mitochondrial RNA Polymerase Is Needed for Activation of the Origin of Light-Strand DNA Replication

(2010) Javier Miralles Fusté et al.   MOLECULAR CELL

Maintenance of respiratory chain function in mouse hearts with severely impaired mtDNA transcription

(2010) Christoph Freyer et al.   NUCLEIC ACIDS RESEARCH

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

Trypanosomes Have Six Mitochondrial DNA Helicases with One Controlling Kinetoplast Maxicircle Replication

(2009) Beiyu Liu et al.   MOLECULAR CELL

Twinkle mutations associated with autosomal dominant progressive external ophthalmoplegia lead to impaired helicase function and in vivo mtDNA replication stalling

(2008) Steffi Goffart et al.   HUMAN MOLECULAR GENETICS

Physiological and Biochemical Defects in Carboxyl-terminal Mutants of Mitochondrial DNA Helicase

(2008) Yuichi Matsushima et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Structure–Function Defects of the TWINKLE Linker Region in Progressive External Ophthalmoplegia

(2008) Jenny A. Korhonen et al.   JOURNAL OF MOLECULAR BIOLOGY

Human mitochondrial RNA polymerase primes lagging-strand DNA synthesis in vitro

(2008) S. Wanrooij et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA