期刊
JOURNAL OF INTERFERON AND CYTOKINE RESEARCH
卷 37, 期 5, 页码 198-206出版社
MARY ANN LIEBERT, INC
DOI: 10.1089/jir.2016.0086
关键词
TREX1; STING; cytosolic DNA sensing; Aicardi-Goutieres syndrome; retinal vasculopathy with cerebral leukodystrophy; STING-associated vasculopathy with onset in infancy
资金
- Rita C. and William P. Clements, Jr. Endowed Scholar Award from UT Southwestern
- U.S. National Institute of Health [AI98569, AR067135]
- Alliance for Lupus Foundation
- Welch Foundation [I-1831]
- Burroughs Wellcome Fund
The innate immune system is the first line of defense against invading pathogens. One important feature of innate immune recognition is self versus nonself discrimination. The selectivity for microbial ligands is achieved through substrate motif specificity, spatial compartmentalization, and functions of negative regulators. Loss-of-function mutations in negative regulators or gain-of-function mutations in drivers of innate immune signaling have been associated with autoimmune diseases such as lupus, rheumatoid arthritis, inflammatory vasculopathy, and a variety of interferonopathies. This review will focus on TREX1 and STING, which are opposing regulators of the cytosolic DNA-sensing pathway. Tremendous effort over the past decade among academic and clinical research groups has elucidated molecular mechanisms underlying immune diseases associated with TREX1 and STING dysfunction. We have also witnessed rapid therapeutic translation of the molecular findings. Several targeted treatment options or druggable candidates are now available for these once incurable diseases. With great enthusiasm from both academia and industry partners, we look forward to seeing the remaining scientific questions answered and, more importantly, the affected patients benefited from these discoveries.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据