Human life within a narrow range: The lethal ups and downs of type I interferons

YJ Crow, JL Casanova - Science immunology, 2024 - science.org
The past 20 years have seen the definition of human monogenic disorders and their
autoimmune phenocopies underlying either defective or enhanced type I interferon (IFN) …

Mechanisms regulating neutrophil responses in immunity, allergy, and autoimmunity

A Özcan, O Boyman - Allergy, 2022 - Wiley Online Library
Neutrophil granulocytes, or neutrophils, are the most abundant circulating leukocytes in
humans and indispensable for antimicrobial immunity, as exemplified in patients with inborn …

Nucleotide metabolism, leukodystrophies, and CNS pathology

F Gavazzi, CD Gonzalez, K Arnold… - Journal of Inherited …, 2024 - Wiley Online Library
The balance between a protective and a destructive immune response can be precarious,
as exemplified by inborn errors in nucleotide metabolism. This class of inherited disorders …

cGAS-STING, inflammasomes and pyroptosis: an overview of crosstalk mechanism of activation and regulation

J Liu, J Zhou, Y Luan, X Li, X Meng, W Liao… - Cell Communication and …, 2024 - Springer
Background Intracellular DNA-sensing pathway cGAS-STING, inflammasomes and
pyroptosis act as critical natural immune signaling axes for microbial infection, chronic …

[HTML][HTML] Immune dysregulation

A Long, A Kleiner, RJ Looney - Journal of Allergy and Clinical Immunology, 2023 - Elsevier
The understanding of immune dysregulation in many different diseases continues to grow.
There is increasing evidence that altered microbiome and gut barrier dysfunction contribute …

Constitutively active Lyn kinase causes a cutaneous small vessel vasculitis and liver fibrosis syndrome

AA de Jesus, G Chen, D Yang, T Brdicka… - Nature …, 2023 - nature.com
Neutrophilic inflammation is a hallmark of many monogenic autoinflammatory diseases;
pathomechanisms that regulate extravasation of damaging immune cells into surrounding …

IFN-signaling gene expression as a diagnostic biomarker for monogenic interferonopathies

LA Adang, R D'Aiello, A Takanohashi, S Woidill… - JCI …, 2024 - pmc.ncbi.nlm.nih.gov
IFN-signaling gene (ISG) expression scores are potential markers of inflammation with
significance from cancer to genetic syndromes. In Aicardi Goutières Syndrome (AGS), a …

Standing on shoulders: interferon research from viral interference to lupus pathogenesis and treatment

MK Crow, M Olferiev, KA Kirou - Arthritis & Rheumatology, 2024 - Wiley Online Library
The discovery of interferon in the 1950s represents much more than the identification of the
first cytokine and the key mediator of antiviral host defense. Defining the molecular nature …

Genetic approaches to study rheumatic diseases and its implications in clinical practice

D Rowczenio, I Aksentijevich - Arthritis & Rheumatology, 2024 - Wiley Online Library
Patients with rare and complex rheumatic diseases (RDs) present with immense clinical
variability inherent to all immunologic diseases. In addition to systemic and organ‐specific …

Innate immunity: a balance between disease and adaption to stress

I Faenza, WL Blalock - Biomolecules, 2022 - mdpi.com
Since first being documented in ancient times, the relation of inflammation with injury and
disease has evolved in complexity and causality. Early observations supported a cause …