Aicardi-Goutières syndrome-associated mutation at ADAR1 gene locus activates innate immune response in mouse brain

Hepatic Involvement in Aicardi-Goutières Syndrome

(2021) Francesco Gavazzi et al.   NEUROPEDIATRICS

ADAR1 mediated regulation of neural crest derived melanocytes and Schwann cell development

(2020) Nadjet Gacem et al.   Nature Communications

cGAS activation causes lupus-like autoimmune disorders in a TREX1 mutant mouse model

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Treatments in Aicardi–Goutières syndrome

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Novel and emerging treatments for Aicardi-Goutières syndrome

(2019) Davide Tonduti et al.   Expert Review of Clinical Immunology

Variable clinical phenotype in two siblings with Aicardi-Goutières syndrome type 6 and a novel mutation in the ADAR gene

(2018) Lisa Schmelzer et al.   EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY

Phenotypic and Molecular Spectrum of Aicardi-Goutières Syndrome: A Study of 24 Patients

(2018) Fuad Al Mutairi et al.   PEDIATRIC NEUROLOGY

RNase H2 Loss in Murine Astrocytes Results in Cellular Defects Reminiscent of Nucleic Acid-Mediated Autoinflammation

(2018) Kareen Bartsch et al.   Frontiers in Immunology

Lack of Trex1 Causes Systemic Autoimmunity despite the Presence of Antiretroviral Drugs

(2017) Martin Achleitner et al.   JOURNAL OF IMMUNOLOGY

Interferon-Stimulated Gene Expression as a Preferred Biomarker for Disease Activity in Aicardi–Goutières Syndrome

(2017) Ben X. Wang et al.   JOURNAL OF INTERFERON AND CYTOKINE RESEARCH

Genetic, Phenotypic, and Interferon Biomarker Status in ADAR1-Related Neurological Disease

(2017) Gillian Rice et al.   NEUROPEDIATRICS

Potential role of CXCL10/CXCR3 signaling in the development of morphine tolerance in periaqueductal gray

(2017) Wei Wang et al.   NEUROPEPTIDES

Ribonuclease H2 mutations induce a cGAS/STING‐dependent innate immune response

(2016) Karen J Mackenzie et al.   EMBO JOURNAL

RNase H2 catalytic core Aicardi-Goutières syndrome–related mutant invokes cGAS–STING innate immune-sensing pathway in mice

(2016) Vladislav Pokatayev et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Loss of Trex1 in Dendritic Cells Is Sufficient To Trigger Systemic Autoimmunity

(2016) Katrin Peschke et al.   JOURNAL OF IMMUNOLOGY

Ultrastructure of Diaschisis Lesions after Traumatic Brain Injury

(2016) Clayton A. Wiley et al.   JOURNAL OF NEUROTRAUMA

Neurologic Phenotypes Associated with Mutations in TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, ADAR1, and IFIH1: Aicardi–Goutières Syndrome and Beyond

(2016) Yanick Crow et al.   NEUROPEDIATRICS

Effects of Aicardi-Goutières syndrome mutations predicted from ADAR-RNA structures

(2016) Andrew J. Fisher et al.   RNA Biology

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

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ADAR1 Prevents Liver Injury from Inflammation and Suppresses Interferon Production in Hepatocytes

(2015) Guoliang Wang et al.   AMERICAN JOURNAL OF PATHOLOGY

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

(2015) Kathleen Pestal et al.   IMMUNITY

Phenotypic Variation in Aicardi–Goutières Syndrome Explained by Cell-Specific IFN-Stimulated Gene Response and Cytokine Release

(2015) Eloy Cuadrado et al.   JOURNAL OF IMMUNOLOGY

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

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

Adenosine Deaminase Acting on RNA 1 Limits RIG-I RNA Detection and Suppresses IFN Production Responding to Viral and Endogenous RNAs

(2014) Shengyong Yang et al.   JOURNAL OF IMMUNOLOGY

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

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

Striking intrafamilial phenotypic variability in Aicardi-Goutières syndrome associated with the recurrent Asian founder mutation inRNASEH2C

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Therapies in Aicardi-Goutières syndrome

(2013) Y. J. Crow et al.   CLINICAL AND EXPERIMENTAL IMMUNOLOGY

The Exonuclease Trex1 Restrains Macrophage Proinflammatory Activation

(2013) S. Pereira-Lopes et al.   JOURNAL OF IMMUNOLOGY

Mouse SAMHD1 Has Antiretroviral Activity and Suppresses a Spontaneous Cell-Intrinsic Antiviral Response

(2013) Rayk Behrendt et al.   Cell Reports

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

(2012) Alevtina Gall et al.   IMMUNITY

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

(2012) Gillian I Rice et al.   NATURE GENETICS

Chemokine mediated neuron–glia communication and aberrant signalling in neuropathic pain states

(2011) Elizabeth Amy Old et al.   CURRENT OPINION IN PHARMACOLOGY

Functions and Regulation of RNA Editing by ADAR Deaminases

(2010) Kazuko Nishikura   Annual Review of Biochemistry

Expanding the phenotypic spectrum of lupus erythematosus in Aicardi-Goutières syndrome

(2010) Georgia Ramantani et al.   ARTHRITIS AND RHEUMATISM

CXCL10/CXCR3 signaling in glia cells differentially affects NMDA-induced cell death in CA and DG neurons of the mouse hippocampus

(2010) Hilmar R.J. van Weering et al.   HIPPOCAMPUS

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

Interferon-Related Transcriptome Alterations in the Cerebrospinal Fluid Cells of Aicardi-Goutières Patients

(2009) Alberto Izzotti et al.   BRAIN PATHOLOGY

Aicardi-Goutieres syndrome

(2009) S. Orcesi et al.   BRITISH MEDICAL BULLETIN

Key role of T cell defects in age-related vulnerability to West Nile virus

(2009) James D. Brien et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Trex1 Prevents Cell-Intrinsic Initiation of Autoimmunity

(2008) Daniel B. Stetson et al.   CELL

Astrocytes produce interferon-alpha and CXCL10, but not IL-6 or CXCL8, in aicardi-Goutières syndrome

(2008) Jane T. Van Heteren et al.   GLIA

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

(2008) Jochen C Hartner et al.   NATURE IMMUNOLOGY