ATAD3 controls mitochondrial cristae structure in mouse muscle, influencing mtDNA replication and cholesterol levels

ATPase-deficient mitochondrial inner membrane protein ATAD3A disturbs mitochondrial dynamics in dominant hereditary spastic paraplegia

(2017) Helen M. Cooper et al.   HUMAN MOLECULAR GENETICS

Recurrent De Novo and Biallelic Variation of ATAD3A , Encoding a Mitochondrial Membrane Protein, Results in Distinct Neurological Syndromes

(2016) Tamar Harel et al.   AMERICAN JOURNAL OF HUMAN GENETICS

CLPP coordinates mitoribosomal assembly through the regulation of ERAL1 levels

(2016) Karolina Szczepanowska et al.   EMBO JOURNAL

Sustained AMPK activation improves muscle function in a mitochondrial myopathy mouse model by promoting muscle fiber regeneration

(2016) Susana Peralta et al.   HUMAN MOLECULAR GENETICS

FGF21 is a biomarker for mitochondrial translation and mtDNA maintenance disorders

(2016) Jenni M. Lehtonen et al.   NEUROLOGY

ER-mitochondria contacts couple mtDNA synthesis with mitochondrial division in human cells

(2016) Samantha C. Lewis et al.   SCIENCE

Mitochondrial Cristae: Where Beauty Meets Functionality

(2016) Sara Cogliati et al.   TRENDS IN BIOCHEMICAL SCIENCES

Optic Atrophy 1 Is Epistatic to the Core MICOS Component MIC60 in Mitochondrial Cristae Shape Control

(2016) Christina Glytsou et al.   Cell Reports

Mitochondrial diseases

(2016) Gráinne S. Gorman et al.   Nature Reviews Disease Primers

Mic60/Mitofilin determines MICOS assembly essential for mitochondrial dynamics and mtDNA nucleoid organization

(2015) H Li et al.   CELL DEATH AND DIFFERENTIATION

Opa1 Overexpression Ameliorates the Phenotype of Two Mitochondrial Disease Mouse Models

(2015) Gabriele Civiletto et al.   Cell Metabolism

Mitochondria-Associated Membrane Formation in Hormone-Stimulated Leydig Cell Steroidogenesis: Role of ATAD3

(2015) Leeyah Issop et al.   ENDOCRINOLOGY

Cholesterol trafficking and distribution

(2015) David B. Iaea et al.   Essays in Biochemistry

Mitochondrial Diseases Part II: Mouse models of OXPHOS deficiencies caused by defects in regulatory factors and other components required for mitochondrial function

(2015) Luisa Iommarini et al.   MITOCHONDRION

Human Mitochondrial DNA-Protein Complexes Attach to a Cholesterol-Rich Membrane Structure

(2015) Joachim M. Gerhold et al.   Scientific Reports

Tissue-Specific Loss of DARS2 Activates Stress Responses Independently of Respiratory Chain Deficiency in the Heart

(2014) Sukru Anil Dogan et al.   Cell Metabolism

Loss of LRPPRC causes ATP synthase deficiency

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

Uniform nomenclature for the mitochondrial contact site and cristae organizing system

(2014) Nikolaus Pfanner et al.   JOURNAL OF CELL BIOLOGY

Catecholamine metabolism drives generation of mitochondrial DNA deletions in dopaminergic neurons

(2013) Johannes F.G. Neuhaus et al.   BRAIN

Mitochondrial Cristae Shape Determines Respiratory Chain Supercomplexes Assembly and Respiratory Efficiency

(2013) Sara Cogliati et al.   CELL

Expression analysis of ATAD3 isoforms in rodent and human cell lines and tissues

(2013) Shuijie Li et al.   GENE

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

(2013) D. Milenkovic et al.   HUMAN MOLECULAR GENETICS

Mitochondrial protein ATPase family, AAA domain containing 3A correlates with radioresistance in glioblastoma

(2013) W.-C. You et al.   NEURO-ONCOLOGY

Atad3 Function Is Essential for Early Post-Implantation Development in the Mouse

(2013) Tobias Goller et al.   PLoS One

Somatic mtDNA Mutation Spectra in the Aging Human Putamen

(2013) Siôn L. Williams et al.   PLoS Genetics

ATAD3, a vital membrane bound mitochondrial ATPase involved in tumor progression

(2012) Shuijie Li et al.   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

Assessment of mitochondrial respiratory chain enzymatic activities on tissues and cultured cells

(2012) Marco Spinazzi et al.   Nature Protocols

Mitochondrial nucleoid interacting proteins support mitochondrial protein synthesis

(2012) J. He et al.   NUCLEIC ACIDS RESEARCH

Structure of the yeast F1Fo-ATP synthase dimer and its role in shaping the mitochondrial cristae

(2012) K. M. Davies et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2011) Yolanda Cámara et al.   Cell Metabolism

Dual Role of Mitofilin in Mitochondrial Membrane Organization and Protein Biogenesis

(2011) Karina von der Malsburg et al.   DEVELOPMENTAL CELL

The mitochondrial contact site complex, a determinant of mitochondrial architecture

(2011) Max Harner et al.   EMBO JOURNAL

Comparative Lipidomic Analysis of Mouse and Human Brain with Alzheimer Disease

(2011) Robin B. Chan et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A mitochondrial-focused genetic interaction map reveals a scaffold-like complex required for inner membrane organization in mitochondria

(2011) Suzanne Hoppins et al.   JOURNAL OF CELL BIOLOGY

Replication stalling by catalytically impaired Twinkle induces mitochondrial DNA rearrangements in cultured cells

(2011) Jaakko L.O. Pohjoismäki et al.   MITOCHONDRION

Macromolecular organization of ATP synthase and complex I in whole mitochondria

(2011) K. M. Davies et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mitochondrial Fusion Is Required for mtDNA Stability in Skeletal Muscle and Tolerance of mtDNA Mutations

(2010) Hsiuchen Chen et al.   CELL

The mtDNA Mutation Spectrum of the Progeroid Polg Mutator Mouse Includes Abundant Control Region Multimers

(2010) Siôn L. Williams et al.   Cell Metabolism

OPA1 links human mitochondrial genome maintenance to mtDNA replication and distribution

(2010) G. Elachouri et al.   GENOME RESEARCH

Topological analysis of ATAD3A insertion in purified human mitochondria

(2010) Arnaud Hubstenberger et al.   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

ATPase family AAA domain-containing 3A is a novel anti-apoptotic factor in lung adenocarcinoma cells

(2010) H.-Y. Fang et al.   JOURNAL OF CELL SCIENCE

The AAA+ ATPase ATAD3A Controls Mitochondrial Dynamics at the Interface of the Inner and Outer Membranes

(2010) B. Gilquin et al.   MOLECULAR AND CELLULAR BIOLOGY

Importing Mitochondrial Proteins: Machineries and Mechanisms

(2009) Agnieszka Chacinska 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

In vivo methylation of mtDNA reveals the dynamics of protein–mtDNA interactions

(2009) Adriana P. Rebelo et al.   NUCLEIC ACIDS RESEARCH

C. elegans ATAD-3 Is Essential for Mitochondrial Activity and Development

(2009) Michael Hoffmann et al.   PLoS One

ATAD 3A and ATAD 3B are distal 1p-located genes differentially expressed in human glioma cell lines and present in vitro anti-oncogenic and chemoresistant properties

(2008) Arnaud Hubstenberger et al.   EXPERIMENTAL CELL RESEARCH

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