Mutations in COPA lead to abnormal trafficking of STING to the Golgi and interferon signaling

Genetic and phenotypic spectrum associated with IFIH1 gain‐of‐function

(2020) Gillian I. Rice et al.   HUMAN MUTATION

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

(2020) Jeonghan Kim et al.   SCIENCE

Type 1 interferonopathy presenting as juvenile idiopathic arthritis with interstitial lung disease: report of a new phenotype

(2020) S. L. N. Clarke et al.   Pediatric Rheumatology

Self-Awareness: Nucleic Acid–Driven Inflammation and the Type I Interferonopathies

(2019) Carolina Uggenti et al.   Annual Review of Immunology

The N-Terminal Domain of cGAS Determines Preferential Association with Centromeric DNA and Innate Immune Activation in the Nucleus

(2019) Matteo Gentili et al.   Cell Reports

Cryo-EM structures of STING reveal its mechanism of activation by cyclic GMP–AMP

(2019) Guijun Shang et al.   NATURE

Autophagy induction via STING trafficking is a primordial function of the cGAS pathway

(2019) Xiang Gui et al.   NATURE

Efficacy and Adverse Events During Janus Kinase Inhibitor Treatment of SAVI Syndrome

(2019) Stefano Volpi et al.   JOURNAL OF CLINICAL IMMUNOLOGY

ARF GTPases and their GEFs and GAPs: concepts and challenges

(2019) Elizabeth Sztul et al.   MOLECULAR BIOLOGY OF THE CELL

STING Polymer Structure Reveals Mechanisms for Activation, Hyperactivation, and Inhibition

(2019) Sabrina L. Ergun et al.   CELL

An unprecedented COPA gene mutation in two patients in the same family: comparative clinical analysis of newly reported patients with other known COPA gene mutations

(2019) Anjali Patwardhan et al.   Pediatric Rheumatology

STING-Associated Vasculopathy with Onset in Infancy in Three Children with New Clinical Aspect and Unsatisfactory Therapeutic Responses to Tofacitinib

(2019) Xiaolei Tang et al.   JOURNAL OF CLINICAL IMMUNOLOGY

Use of ruxolitinib in COPA syndrome manifesting as life-threatening alveolar haemorrhage

(2019) Marie-Louise Frémond et al.   THORAX

Distinct interferon signatures and cytokine patterns define additional systemic autoinflammatory diseases

(2019) Adriana A. de Jesus et al.   JOURNAL OF CLINICAL INVESTIGATION

The binding of TBK1 to STING requires exocytic membrane traffic from the ER

(2018) Emari Ogawa et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Type I interferon pathway activation in COPA syndrome

(2018) Stefano Volpi et al.   CLINICAL IMMUNOLOGY

Formation of COPI-coated vesicles at a glance

(2018) Eric C. Arakel et al.   JOURNAL OF CELL SCIENCE

JAK1/2 inhibition with baricitinib in the treatment of autoinflammatory interferonopathies

(2018) Gina A. Montealegre Sanchez et al.   JOURNAL OF CLINICAL INVESTIGATION

NONO Detects the Nuclear HIV Capsid to Promote cGAS-Mediated Innate Immune Activation

(2018) Xavier Lahaye et al.   CELL

Mitochondrial double-stranded RNA triggers antiviral signalling in humans

(2018) Ashish Dhir et al.   NATURE

Efficacy of JAK1/2 inhibition in the treatment of chilblain lupus due to TREX1 deficiency

(2018) Coralie Briand et al.   ANNALS OF THE RHEUMATIC DISEASES

Expanding the phenotype of COPA syndrome: a kindred with typical and atypical features

(2018) Angelo M Taveira-DaSilva et al.   JOURNAL OF MEDICAL GENETICS

The Ca2+ sensor STIM1 regulates the type I interferon response by retaining the signaling adaptor STING at the endoplasmic reticulum

(2018) Sonal Srikanth et al.   NATURE IMMUNOLOGY

Disease-associated mutations identify a novel region in human STING necessary for the control of type I interferon signaling

(2017) Isabelle Melki et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

Intrinsic antiproliferative activity of the innate sensor STING in T lymphocytes

(2017) Silvia Cerboni et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Detection of interferon alpha protein reveals differential levels and cellular sources in disease

(2017) Mathieu P. Rodero et al.   JOURNAL OF EXPERIMENTAL MEDICINE

cGAS surveillance of micronuclei links genome instability to innate immunity

(2017) Karen J. Mackenzie et al.   NATURE

Imaging findings of Copa syndrome in a 12-year-old boy

(2017) Razan Noorelahi et al.   PEDIATRIC RADIOLOGY

STING is an essential mediator of the Ku70-mediated production of IFN-λ1 in response to exogenous DNA

(2017) Hongyan Sui et al.   Science Signaling

Trafficking-Mediated STING Degradation Requires Sorting to Acidified Endolysosomes and Can Be Targeted to Enhance Anti-tumor Response

(2017) Vijay K. Gonugunta et al.   Cell Reports

9Å structure of the COPI coat reveals that the Arf1 GTPase occupies two contrasting molecular environments

(2017) Svetlana O Dodonova et al.   eLife

STING is an essential mediator of the Ku70-mediated production of IFN-λ1 in response to exogenous DNA

(2017) Hongyan Sui et al.   Science Signaling

Brief Report: Blockade of TANK-Binding Kinase 1/IKKɛ Inhibits Mutant Stimulator of Interferon Genes (STING)-Mediated Inflammatory Responses in Human Peripheral Blood Mononuclear Cells

(2017) Marie-Louise Frémond et al.   Arthritis & Rheumatology

AIRE-Deficient Patients Harbor Unique High-Affinity Disease-Ameliorating Autoantibodies

(2016) Steffen Meyer et al.   CELL

Efficacy of the Janus kinase 1/2 inhibitor ruxolitinib in the treatment of vasculopathy associated with TMEM173 -activating mutations in 3 children

(2016) Marie-Louise Frémond et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

Copa Syndrome: a Novel Autosomal Dominant Immune Dysregulatory Disease

(2016) Timothy J. Vece et al.   JOURNAL OF CLINICAL IMMUNOLOGY

Assessment of Type I Interferon Signaling in Pediatric Inflammatory Disease

(2016) Gillian I. Rice et al.   JOURNAL OF CLINICAL IMMUNOLOGY

Human USP18 deficiency underlies type 1 interferonopathy leading to severe pseudo-TORCH syndrome

(2016) Marije E.C. Meuwissen et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Activation of STING requires palmitoylation at the Golgi

(2016) Kojiro Mukai et al.   Nature Communications

STING Activation by Translocation from the ER Is Associated with Infection and Autoinflammatory Disease

(2015) Nicole Dobbs et al.   Cell Host & Microbe

DNA Damage Primes the Type I Interferon System via the Cytosolic DNA Sensor STING to Promote Anti-Microbial Innate Immunity

(2015) Anetta Härtlova et al.   IMMUNITY

Coordinated regulation of bidirectional COPI transport at the Golgi by CDC42

(2015) Seung-Yeol Park et al.   NATURE

COPA mutations impair ER-Golgi transport and cause hereditary autoimmune-mediated lung disease and arthritis

(2015) Levi B Watkin et al.   NATURE GENETICS

Phosphorylation of innate immune adaptor proteins MAVS, STING, and TRIF induces IRF3 activation

(2015) S. Liu et al.   SCIENCE

Inherited STING-activating mutation underlies a familial inflammatory syndrome with lupus-like manifestations

(2014) Nadia Jeremiah et al.   JOURNAL OF CLINICAL INVESTIGATION

Human intracellular ISG15 prevents interferon-α/β over-amplification and auto-inflammation

(2014) Xianqin Zhang et al.   NATURE

Gain-of-function mutations in IFIH1 cause a spectrum of human disease phenotypes associated with upregulated type I interferon signaling

(2014) Gillian I Rice et al.   NATURE GENETICS

Activated STING in a Vascular and Pulmonary Syndrome

(2014) Yin Liu et al.   NEW ENGLAND JOURNAL OF MEDICINE

Rules for the recognition of dilysine retrieval motifs by coatomer

(2013) Wenfu Ma et al.   EMBO JOURNAL

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

Organization of the ER–Golgi interface for membrane traffic control

(2013) Federica Brandizzi et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Stapled Golgi cisternae remain in place as cargo passes through the stack

(2013) Gregory Lavieu et al.   eLife

Inter-Golgi transport mediated by COPI-containing vesicles carrying small cargoes

(2013) Patrina A Pellett et al.   eLife

Structural Analysis of the STING Adaptor Protein Reveals a Hydrophobic Dimer Interface and Mode of Cyclic di-GMP Binding

(2012) Songying Ouyang 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

Cyclic GMP-AMP Synthase Is a Cytosolic DNA Sensor That Activates the Type I Interferon Pathway

(2012) L. Sun et al.   SCIENCE

STING is a direct innate immune sensor of cyclic di-GMP

(2011) Dara L. Burdette et al.   NATURE

COPI Budding within the Golgi Stack

(2011) V. Popoff et al.   Cold Spring Harbor Perspectives in Biology

Induction of cortical endoplasmic reticulum by dimerization of a coatomer-binding peptide anchored to endoplasmic reticulum membranes

(2010) G. Lavieu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Early endosomes and endosomal coatomer are required for autophagy

(2009) Minoo Razi et al.   JOURNAL OF CELL BIOLOGY