Mitochondrial cristae architecture protects against mtDNA release and inflammation

Disuse-associated loss of the protease LONP1 in muscle impairs mitochondrial function and causes reduced skeletal muscle mass and strength

(2022) Zhisheng Xu et al.   Nature Communications

In-frame deletion in canine PITRM1 is associated with a severe early-onset epilepsy, mitochondrial dysfunction and neurodegeneration

(2021) Marjo K. Hytönen et al.   HUMAN GENETICS

Loss of Mitochondrial Protease CLPP Activates Type I IFN Responses through the Mitochondrial DNA–cGAS–STING Signaling Axis

(2021) Sylvia Torres-Odio et al.   JOURNAL OF IMMUNOLOGY

Glycerol-3-phosphate biosynthesis regenerates cytosolic NAD+ to alleviate mitochondrial disease

(2021) Shanshan Liu et al.   Cell Metabolism

Enhanced cGAS-STING–dependent interferon signaling associated with mutations in ATAD3A

(2021) Alice Lepelley et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Cytoplasmic DNA: sources, sensing, and role in aging and disease

(2021) Karl N. Miller et al.   CELL

Quantitative high-confidence human mitochondrial proteome and its dynamics in cellular context

(2021) Marcel Morgenstern et al.   Cell Metabolism

Threshold of heteroplasmic truncating MT-ATP6 mutation in reprogramming, Notch hyperactivation and motor neuron metabolism

(2021) Sebastian Kenvin et al.   HUMAN MOLECULAR GENETICS

ATAD3A has a scaffolding role regulating mitochondria inner membrane structure and protein assembly

(2021) Tania Arguello et al.   Cell Reports

Shaping the mitochondrial inner membrane in health and disease

(2020) L. Colina‐Tenorio et al.   JOURNAL OF INTERNAL MEDICINE

VDAC oligomers form mitochondrial pores to release mtDNA fragments and promote lupus-like disease

(2020) Jeonghan Kim et al.   SCIENCE

Mitochondrial DNA in inflammation and immunity

(2020) Joel S Riley et al.   EMBO REPORTS

Loss of CHCHD2 and CHCHD10 activates OMA1 peptidase to disrupt mitochondrial cristae phenocopying patient mutations

(2020) Yi-Ting Liu et al.   HUMAN MOLECULAR GENETICS

Barrier-to-Autointegration Factor 1 Protects against a Basal cGAS-STING Response

(2020) Hongming Ma et al.   mBio

FBXL 4 deficiency increases mitochondrial removal by autophagy

(2020) David Alsina et al.   EMBO Molecular Medicine

Altered MICOS Morphology and Mitochondrial Ion Homeostasis Contribute to Poly(GR) Toxicity Associated with C9-ALS/FTD

(2020) Shuangxi Li et al.   Cell Reports

Mitochondrial Quality Control and Restraining Innate Immunity

(2020) Andrew T. Moehlman et al.   Annual Review of Cell and Developmental Biology

TDP-43 Triggers Mitochondrial DNA Release via mPTP to Activate cGAS/STING in ALS

(2020) Chien-Hsiung Yu et al.   CELL

Cristae Membrane Dynamics – A Paradigm Change

(2020) Arun Kumar Kondadi et al.   TRENDS IN CELL BIOLOGY

Loss of MTX2 causes mandibuloacral dysplasia and links mitochondrial dysfunction to altered nuclear morphology

(2020) Sahar Elouej et al.   Nature Communications

ROMO1 is a constituent of the human presequence translocase required for YME1L protease import

(2019) Frank Richter et al.   JOURNAL OF CELL BIOLOGY

Mutations in TIMM50 cause severe mitochondrial dysfunction by targeting key aspects of mitochondrial physiology

(2019) Frederic Tort et al.   HUMAN MUTATION

C9ORF72-ALS/FTD-associated poly(GR) binds Atp5a1 and compromises mitochondrial function in vivo

(2019) So Yoen Choi et al.   NATURE NEUROSCIENCE

TDP-43 induces mitochondrial damage and activates the mitochondrial unfolded protein response

(2019) Peng Wang et al.   PLoS Genetics

Mitochondrial Damage and Activation of the STING Pathway Lead to Renal Inflammation and Fibrosis

(2019) Ki Wung Chung et al.   Cell Metabolism

USMG5 Ashkenazi Jewish founder mutation impairs mitochondrial complex V dimerization and ATP synthesis

(2018) Emanuele Barca et al.   HUMAN MOLECULAR GENETICS

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

(2018) Susana Peralta et al.   JOURNAL OF CELL SCIENCE

The cGAS–cGAMP–STING pathway connects DNA damage to inflammation, senescence, and cancer

(2018) Tuo Li et al.   JOURNAL OF EXPERIMENTAL MEDICINE

PINK1 Phosphorylates MIC60/Mitofilin to Control Structural Plasticity of Mitochondrial Crista Junctions

(2018) Pei-I Tsai et al.   MOLECULAR CELL

ISG15 in antiviral immunity and beyond

(2018) Yi-Chieh Perng et al.   NATURE REVIEWS MICROBIOLOGY

BAK/BAX macropores facilitate mitochondrial herniation and mtDNA efflux during apoptosis

(2018) Kate McArthur et al.   SCIENCE

Detection of Microbial Infections Through Innate Immune Sensing of Nucleic Acids

(2018) Xiaojun Tan et al.   Annual Review of Microbiology

Mitochondrial inner membrane permeabilisation enables mtDNA release during apoptosis

(2018) Joel S Riley et al.   EMBO JOURNAL

The role of mitochondria in aging

(2018) Ji Yong Jang et al.   JOURNAL OF CLINICAL INVESTIGATION

Mitochondrial double-stranded RNA triggers antiviral signalling in humans

(2018) Ashish Dhir et al.   NATURE

Parkin and PINK1 mitigate STING-induced inflammation

(2018) Danielle A. Sliter et al.   NATURE

Loss of MICOS complex integrity and mitochondrial damage, but not TDP-43 mitochondrial localisation, are likely associated with severity of CHCHD10-related diseases

(2018) Emmanuelle C. Genin et al.   NEUROBIOLOGY OF DISEASE

FUS interacts with ATP synthase beta subunit and induces mitochondrial unfolded protein response in cellular and animal models

(2018) Jianwen Deng et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Genome-scale CRISPR-Cas9 knockout and transcriptional activation screening

(2017) Julia Joung et al.   Nature Protocols

Loss of Parkinson’s disease-associated protein CHCHD2 affects mitochondrial crista structure and destabilizes cytochrome c

(2017) Hongrui Meng et al.   Nature Communications

Changes of mitochondrial ultrastructure and function during ageing in mice and Drosophila

(2017) Tobias Brandt et al.   eLife

Evolution and structural organization of the mitochondrial contact site (MICOS) complex and the mitochondrial intermembrane space bridging (MIB) complex

(2016) Martijn A. Huynen et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

StarD7 Protein Deficiency Adversely Affects the Phosphatidylcholine Composition, Respiratory Activity, and Cristae Structure of Mitochondria

(2016) Yasuhiro Horibata et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Optimized sgRNA design to maximize activity and minimize off-target effects of CRISPR-Cas9

(2016) John G Doench et al.   NATURE BIOTECHNOLOGY

The inhibition of TDP-43 mitochondrial localization blocks its neuronal toxicity

(2016) Wenzhang Wang et al.   NATURE MEDICINE

Mitochondrial Cristae: Where Beauty Meets Functionality

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

QIL1 mutation causes MICOS disassembly and early onset fatal mitochondrial encephalopathy with liver disease

(2016) Virginia Guarani et al.   eLife

Mitochondrial DNA stress primes the antiviral innate immune response

(2015) A. Phillip West et al.   NATURE

Targeting mitochondria metabolism for cancer therapy

(2015) Samuel E Weinberg et al.   Nature Chemical Biology

MitoCarta2.0: an updated inventory of mammalian mitochondrial proteins

(2015) Sarah E. Calvo et al.   NUCLEIC ACIDS RESEARCH

Apoptotic Caspases Suppress mtDNA-Induced STING-Mediated Type I IFN Production

(2014) Michael J. White et al.   CELL

Apoptotic Caspases Prevent the Induction of Type I Interferons by Mitochondrial DNA

(2014) Anthony Rongvaux et al.   CELL

CRISPR-Cas9 Knockin Mice for Genome Editing and Cancer Modeling

(2014) Randall J. Platt et al.   CELL

Thei-AAA protease YME1L and OMA1 cleave OPA1 to balance mitochondrial fusion and fission

(2014) Ruchika Anand et al.   JOURNAL OF CELL BIOLOGY

Improved vectors and genome-wide libraries for CRISPR screening

(2014) Neville E Sanjana et al.   NATURE METHODS

The Hallmarks of Aging

(2013) Carlos López-Otín et al.   CELL

A bright monomeric green fluorescent protein derived from Branchiostoma lanceolatum

(2013) Nathan C Shaner et al.   NATURE METHODS

Age-dependent dissociation of ATP synthase dimers and loss of inner-membrane cristae in mitochondria

(2013) B. Daum et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

STAR: ultrafast universal RNA-seq aligner

(2012) Alexander Dobin et al.   BIOINFORMATICS

Shaping the role of mitochondria in the pathogenesis of Huntington's disease

(2012) Veronica Costa et al.   EMBO JOURNAL

Impaired mitochondrial biogenesis, defective axonal transport of mitochondria, abnormal mitochondrial dynamics and synaptic degeneration in a mouse model of Alzheimer's disease

(2011) M. J. Calkins et al.   HUMAN MOLECULAR GENETICS

Mitochondrial fission and cristae disruption increase the response of cell models of Huntington's disease to apoptotic stimuli

(2010) Veronica Costa et al.   EMBO Molecular Medicine

TheN-Ethyl-N-Nitrosourea-InducedGoldenticketMouse Mutant Reveals an Essential Function ofStingin theIn VivoInterferon Response toListeria monocytogenesand Cyclic Dinucleotides

(2010) John-Demian Sauer et al.   INFECTION AND IMMUNITY

TMEM70 mutations cause isolated ATP synthase deficiency and neonatal mitochondrial encephalocardiomyopathy

(2008) Alena Čížková et al.   NATURE GENETICS

The amyloid  -peptide is imported into mitochondria via the TOM import machinery and localized to mitochondrial cristae

(2008) C. A. Hansson Petersen et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA