How STING causes inflammation in SAVI, a childhood autoinflammatory disease
Mechanisms of STING-associated autoinflammation
This project looks at how a molecule called STING causes inflammation and immune problems in people with SAVI using specially engineered mouse and cell models.
Quick facts
| Grant type | R01 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | University of Pennsylvania NIH-funded |
| Lab location | 1 site (Philadelphia, United States) |
| Project ID | NIH-11250999 on NIH RePORTER |
What this research studies
Researchers will use genetically engineered mice that carry the same STING gain-of-function changes found in SAVI and turn the mutant STING on in specific cell types to see which cells drive disease. The team will study macrophages, innate lymphoid cells, and radioresistant cells to measure cytokine release, immune cell changes, and tissue inflammation. They will pay special attention to type II interferon (IFN-γ) signaling because prior work showed it drives the disease in this model. Findings aim to reveal which cell types and pathways are responsible for lung, skin, and vascular symptoms.
Who could benefit from this research
Good fit: People with SAVI or known STING gain-of-function mutations and patients with related interferon-driven autoinflammatory conditions would be most relevant to these findings and future trials.
Not a fit: Patients with unrelated causes of inflammation or immune disease that are not driven by STING or IFN-γ signaling are unlikely to benefit directly from this research.
Why it matters
Potential benefit: If successful, this work could point to specific immune cells or the IFN-γ pathway as targets for treatments to reduce inflammation and organ damage in SAVI.
How similar studies have performed: The investigators' prior mouse studies showed that IFN-γ, not type I IFN, drives STING-related disease, so this project builds on those encouraging mechanistic findings with newer cell-specific models.
Where this research is happening
Philadelphia, United States
- University of Pennsylvania — Philadelphia, United States (Active)
Researchers
- Principal investigator: Miner, Jonathan J — University of Pennsylvania
- Study coordinator: Miner, Jonathan J
About this research
- This is an active NIH-funded research project — typically early-stage science, not a clinical trial accepting patient enrollment.
- Some NIH-funded labs run parallel clinical studies or seek volunteers for related work. To check, contact the principal investigator or institution listed above.
- For full project details, budget, and progress reports, visit the official NIH RePORTER page below.