Bennett Lab

Children's Hosp of Philadelphia

Philadelphia · United States

NIH-funded
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NIH R01 · 2024

Interactions between diverse brain cell types drive Aicardi-Goutieres neuropathology

ABSTRACT Aicardi Goutières (AGS) is a severe autoinflammatory disease that predominantly affects the brain, leading to severe cognitive and physical disabilities. Although this disease is genetically heterogeneous, all genotypes lead to multi-system excessive type 1 interferon (IFN) activity. How the systemic inflammatory response in AGS leads to predominant central nervous system (CNS) injury is not entirely understood, which limits development of effective and targeted therapies for this destructive disease. In this study proposal, we aim to uncover the “driver” cell in either the peripheral or CNS immune system or at brain vascular interfaces that converts genetic mutations into…

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