Restricting retrotransposons: a review

JL Goodier - Mobile DNA, 2016 - Springer
Retrotransposons have generated about 40% of the human genome. This review examines
the strategies the cell has evolved to coexist with these genomic “parasites”, focussing on …

Long 3′ UTRs predispose neurons to inflammation by promoting immunostimulatory double-stranded RNA formation

TJ Dorrity, H Shin, KA Wiegand, J Aruda… - Science …, 2023 - science.org
Loss of RNA homeostasis underlies numerous neurodegenerative and neuroinflammatory
diseases. However, the molecular mechanisms that trigger neuroinflammation are poorly …

Self-awareness: Nucleic acid–driven inflammation and the type I interferonopathies

C Uggenti, A Lepelley, YJ Crow - Annual review of immunology, 2019 - annualreviews.org
Recognition of foreign nucleic acids is the primary mechanism by which a type I interferon–
mediated antiviral response is triggered. Given that human cells are replete with DNA and …

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

KJ Mackenzie, P Carroll, L Lettice… - The EMBO …, 2016 - embopress.org
Aicardi–Goutières syndrome (AGS) provides a monogenic model of nucleic acid‐mediated
inflammation relevant to the pathogenesis of systemic autoimmunity. Mutations that impair …

Astrocytes are central in the pathomechanisms of vanishing white matter

S Dooves, M Bugiani, NL Postma… - The Journal of …, 2016 - Am Soc Clin Investig
Vanishing white matter (VWM) is a fatal leukodystrophy that is caused by mutations in genes
encoding subunits of eukaryotic translation initiation factor 2B (eIF2B). Disease onset and …

Severe type I interferonopathy and unrestrained interferon signaling due to a homozygous germline mutation in STAT2

CJA Duncan, BJ Thompson, R Chen, GI Rice… - Science …, 2019 - science.org
Excessive type I interferon (IFNα/β) activity is implicated in a spectrum of human disease, yet
its direct role remains to be conclusively proven. We investigated two siblings with severe …

Novel and emerging treatments for Aicardi-Goutières syndrome

D Tonduti, E Fazzi, R Badolato… - Expert Review of Clinical …, 2020 - Taylor & Francis
ABSTRACT Introduction: Aicardi-Goutières syndrome (AGS) is the prototype of the type I
interferonopathies, a new heterogeneous group of autoinflammatory disorders in which type …

Cellular and molecular mechanisms of immune dysregulation and autoimmunity

G Azizi, MR Pouyani, H Abolhassani, L Sharifi… - Cellular …, 2016 - Elsevier
Primary immunodeficiencies (PIDs) constitute a large group of rare disorders that affect the
function of the immune system. A specific group of PIDs entitled “diseases of immune …

International union of basic and clinical pharmacology. XCVI. Pattern recognition receptors in health and disease

CE Bryant, S Orr, B Ferguson, MF Symmons… - Pharmacological …, 2015 - Elsevier
Since the discovery of Toll, in the fruit fly Drosophila melanogaster, as the first described
pattern recognition receptor (PRR) in 1996, many families of these receptors have been …

Astrocytes, an active player in Aicardi–Goutières syndrome

S Sase, A Takanohashi, A Vanderver… - Brain …, 2018 - Wiley Online Library
Aicardi–Goutières syndrome (AGS) is an early‐onset, autoimmune and genetically
heterogeneous disorder with severe neurologic injury. Molecular studies have established …