Metabolism, infection and immunity in inborn errors of metabolism

摘要：我的翻译研究项目重点是以线粒体疾病为模型系统的免疫代谢。线粒体疾病患者经历了由感染引起的急性代谢不稳定和器官功能障碍的威胁生命的事件，称为代谢失代偿。我们的目标是了解感染过程中的免疫激活是如何对这种病理生理做出贡献的。这一系列研究也对脓毒症期间的器官功能障碍有影响，败血症是重症监护病房的常见问题。鉴于感染在线粒体疾病患者中的重要性，我们也希望了解线粒体代谢在免疫细胞功能中的作用。许多参与线粒体代谢的酶也在淋巴细胞中表达，并与疾病相关。这些研究不仅有助于深入了解线粒体疾病患者的免疫功能，还有助于确定线粒体在正常免疫细胞功能中的作用。

Summary: My translational research program focuses on immunometabolism using mitochondrial disease as a model system. Patients with mitochondrial disease experience life threatening episodes of acute metabolic instability and organ dysfunction brought about by infection termed metabolic decompensation. Our goal is to understand how immune activation during infection contributes to this pathophysiology. This line of research also has implications for organ dysfunction during sepsis, a common problem in the intensive care unit. Since infection is important in patients with mitochondrial disease, we also wish to understand the role of mitochondrial metabolism in immune cell function. Many enzymes involved in mitochondrial metabolism are also expressed in lymphocytes, and have associated diseases. These studies will not only provide insight into immune function in mitochondrial disease patients, but also help define the role of the mitochondria in normal immune cell function.