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Updated: Nov 19, 2025

Visualizing Lung Cellular Adaptations during Combined Ozone and LPS Induced Murine Acute Lung Injury

Visualizing Lung Cellular Adaptations during Combined Ozone and LPS Induced Murine Acute Lung Injury

Published on: March 21, 2021

STING-Mediated Lung Inflammation and Beyond.

Marie-Louise Frémond^1,2, Yanick J Crow^3,4

¹Université de Paris, Imagine Institute, Laboratory of Neurogenetics and Neuroinflammation, 24 boulevard du Montparnasse, F-75015, Paris, France. marie-louise.fremond@aphp.fr.

Journal of Clinical Immunology

|February 3, 2021

Summary

This summary is machine-generated.

Type I interferonopathies, including SAVI and COPA syndrome, involve immune system overactivation and lung inflammation. Understanding STING protein interactions clarifies disease mechanisms and guides treatment for these rare genetic disorders.

Keywords:

COPA SAVI STING alveolar hemorrhage interstitial lung disease lung inflammation

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Area of Science:

Immunology
Genetics
Pulmonology

Background:

Mendelian autoinflammatory diseases, or type I interferonopathies, are linked to constitutive type I interferon pathway activation.
STING-associated vasculopathy with onset in infancy (SAVI) and COPA syndrome are emerging interferonopathies with significant lung inflammation, morbidity, and mortality.
These conditions share clinical and histopathological features, with COPA syndrome exhibiting notable non-penetrance.

Purpose of the Study:

To review the expanding phenotypes of SAVI and COPA syndrome.
To highlight commonalities and distinctions between SAVI and COPA syndrome.
To discuss recent advances in STING biology and their therapeutic implications.

Main Methods:

Review of current literature on SAVI and COPA syndrome.
Analysis of STING protein's role in interferonopathies.
Correlation of genetic mutations with clinical manifestations.

Main Results:

SAVI arises from STING1 gain-of-function mutations; COPA syndrome from COPA mutations affecting protein transport.
COPA's role in STING trafficking provides a molecular link explaining phenotypic overlap.
STING activation is implicated in other inflammatory lung diseases beyond these Mendelian disorders.

Conclusions:

SAVI and COPA syndrome represent a spectrum of type I interferonopathies with shared inflammatory lung pathology.
Understanding STING and COPA interactions is crucial for diagnosing and managing these conditions.
STING-mediated DNA sensing pathways are relevant to broader inflammatory lung diseases.