Protein kinase R and the integrated stress response drive immunopathology caused by mutations in the RNA deaminase ADAR1

A phenolic small molecule inhibitor of RNase L prevents cell death from ADAR1 deficiency

(2020) Salima Daou et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

eIF2B activator prevents neurological defects caused by a chronic integrated stress response

(2019) Yao Liang Wong et al.   eLife

Human ADAR1 Prevents Endogenous RNA from Triggering Translational Shutdown

(2018) Hachung Chung et al.   CELL

Breaching Self-Tolerance to Alu Duplex RNA Underlies MDA5-Mediated Inflammation

(2018) Sadeem Ahmad et al.   CELL

Binding of ISRIB reveals a regulatory site in the nucleotide exchange factor eIF2B

(2018) Alisa F. Zyryanova et al.   SCIENCE

Structure of the nucleotide exchange factor eIF2B reveals mechanism of memory-enhancing molecule

(2018) Jordan C. Tsai et al.   SCIENCE

Loss of ADAR1 in tumours overcomes resistance to immune checkpoint blockade

(2018) Jeffrey J. Ishizuka et al.   NATURE

Identification of ADAR1 adenosine deaminase dependency in a subset of cancer cells

(2018) Hugh S. Gannon et al.   Nature Communications

Ribonuclease L mediates the cell-lethal phenotype of double-stranded RNA editing enzyme ADAR1 deficiency in a human cell line

(2017) Yize Li et al.   eLife

The AIM2-like Receptors Are Dispensable for the Interferon Response to Intracellular DNA

(2016) Elizabeth E. Gray et al.   IMMUNITY

Editing of Cellular Self-RNAs by Adenosine Deaminase ADAR1 Suppresses Innate Immune Stress Responses

(2016) Cyril X. George et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Harnessing the integrated stress response for the treatment of multiple sclerosis

(2016) Sharon W Way et al.   LANCET NEUROLOGY

PKR Transduces MDA5-Dependent Signals for Type I IFN Induction

(2016) Alissa M. Pham et al.   PLoS Pathogens

Characterization of human disease phenotypes associated with mutations inTREX1,RNASEH2A,RNASEH2B,RNASEH2C,SAMHD1,ADAR, andIFIH1

(2015) Yanick J. Crow et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

Isoforms of RNA-Editing Enzyme ADAR1 Independently Control Nucleic Acid Sensor MDA5-Driven Autoimmunity and Multi-organ Development

(2015) Kathleen Pestal et al.   IMMUNITY

Cutting Edge: cGAS Is Required for Lethal Autoimmune Disease in the Trex1-Deficient Mouse Model of Aicardi–Goutières Syndrome

(2015) Elizabeth E. Gray et al.   JOURNAL OF IMMUNOLOGY

Activation of cyclic GMP-AMP synthase by self-DNA causes autoimmune diseases

(2015) Daxing Gao et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

RNA editing by ADAR1 prevents MDA5 sensing of endogenous dsRNA as nonself

(2015) B. J. Liddicoat et al.   SCIENCE

Mutations in a translation initiation factor identify the target of a memory-enhancing compound

(2015) Y. Sekine et al.   SCIENCE

A Critical Role for PKR Complexes with TRBP in Immunometabolic Regulation and eIF2α Phosphorylation in Obesity

(2015) Takahisa Nakamura et al.   Cell Reports

The small molecule ISRIB reverses the effects of eIF2α phosphorylation on translation and stress granule assembly

(2015) Carmela Sidrauski et al.   eLife

The Innate Immune Sensor LGP2 Activates Antiviral Signaling by Regulating MDA5-RNA Interaction and Filament Assembly

(2014) Annie M. Bruns et al.   MOLECULAR CELL

Easy quantitative assessment of genome editing by sequence trace decomposition

(2014) Eva K. Brinkman et al.   NUCLEIC ACIDS RESEARCH

The RNA-Editing Enzyme ADAR1 Controls Innate Immune Responses to RNA

(2014) Niamh M. Mannion et al.   Cell Reports

Identification of an LGP2-associated MDA5 agonist in picornavirus-infected cells

(2014) Safia Deddouche et al.   eLife

Cytosolic Sensing of Viruses

(2013) Delphine Goubau et al.   IMMUNITY

A type I interferon signature identifies bilateral striatal necrosis due to mutations inADAR1

(2013) John H Livingston et al.   JOURNAL OF MEDICAL GENETICS

Assessment of interferon-related biomarkers in Aicardi-Goutières syndrome associated with mutations in TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, and ADAR: a case-control study

(2013) Gillian I Rice et al.   LANCET NEUROLOGY

Structural mechanism of cytosolic DNA sensing by cGAS

(2013) Filiz Civril et al.   NATURE

Pharmacological brake-release of mRNA translation enhances cognitive memory

(2013) Carmela Sidrauski et al.   eLife

The RIG-I-like Receptor LGP2 Controls CD8+ T Cell Survival and Fitness

(2012) Mehul S. Suthar et al.   IMMUNITY

Autoimmunity Initiates in Nonhematopoietic Cells and Progresses via Lymphocytes in an Interferon-Dependent Autoimmune Disease

(2012) Alevtina Gall et al.   IMMUNITY

Dyschromatosis symmetrica hereditaria

(2012) Masahiro Hayashi et al.   JOURNAL OF DERMATOLOGY

Mutations in ADAR1 cause Aicardi-Goutières syndrome associated with a type I interferon signature

(2012) Gillian I Rice et al.   NATURE GENETICS

The Mechanism of Eukaryotic Translation Initiation: New Insights and Challenges

(2012) A. G. Hinnebusch et al.   Cold Spring Harbor Perspectives in Biology

Autoimmune Disease Risk Variant of IFIH1 Is Associated with Increased Sensitivity to IFN-  and Serologic Autoimmunity in Lupus Patients

(2011) T. Robinson et al.   JOURNAL OF IMMUNOLOGY

Protein Kinase R Contributes to Immunity against Specific Viruses by Regulating Interferon mRNA Integrity

(2010) Oliver Schulz et al.   Cell Host & Microbe

The Oligoadenylate Synthetase Family: An Ancient Protein Family with Multiple Antiviral Activities

(2010) Helle Kristiansen et al.   JOURNAL OF INTERFERON AND CYTOKINE RESEARCH

Alu Sequences in Undifferentiated Human Embryonic Stem Cells Display High Levels of A-to-I RNA Editing

(2010) Sivan Osenberg et al.   PLoS One

RNA editing enzyme adenosine deaminase is a restriction factor for controlling measles virus replication that also is required for embryogenesis

(2010) S. V. Ward et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

LGP2 is a positive regulator of RIG-I- and MDA5-mediated antiviral responses

(2010) T. Satoh et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Rare Variants of IFIH1, a Gene Implicated in Antiviral Responses, Protect Against Type 1 Diabetes

(2009) S. Nejentsev et al.   SCIENCE

RNA adenosine deaminase ADAR1 deficiency leads to increased activation of protein kinase PKR and reduced vesicular stomatitis virus growth following interferon treatment

(2009) Zhiqun Li et al.   VIROLOGY

Trex1 Prevents Cell-Intrinsic Initiation of Autoimmunity

(2008) Daniel B. Stetson et al.   CELL

Protein kinase R reveals an evolutionary model for defeating viral mimicry

(2008) Nels C. Elde et al.   NATURE

ADAR1 is essential for the maintenance of hematopoiesis and suppression of interferon signaling

(2008) Jochen C Hartner et al.   NATURE IMMUNOLOGY