Smith Nathan, Wilson Mark A
Department of Biochemistry and the Redox Biology Center, University of Nebraska, Lincoln, NE, 68588, USA.
Adv Exp Med Biol. 2017;1037:5-24. doi: 10.1007/978-981-10-6583-5_2.
The DJ-1 (also called the DJ-1/PfpI, ThiJ/PfpI, or DJ-1/ThiJ/PfpI) superfamily is a structural and functional diverse group of proteins that are present in most organisms. Many of these proteins remain poorly characterized at the biochemical level, but include some known chaperones, proteases, and various stress response proteins that remain mechanistically mysterious. This chapter outlines what is known from a structural perspective about the cellular and biochemical functions of many of these proteins from distinct clades of the superfamily in several organisms. In humans, DJ-1 appears to function primarily as a redox-responsive protein that may act as a sensor for imbalances in cellular redox state. Because mutations in human DJ-1 cause certain types of heritable Parkinson's disease, the role of oxidative posttranslational modifications and pathogenic mutations in human DJ-1 is emphasized in the latter sections of this chapter.
DJ-1（也称为DJ-1/PfpI、ThiJ/PfpI或DJ-1/ThiJ/PfpI）超家族是一类结构和功能多样的蛋白质，存在于大多数生物体中。这些蛋白质中的许多在生化水平上仍未得到充分表征，但包括一些已知的伴侣蛋白、蛋白酶和各种应激反应蛋白，其作用机制仍然神秘。本章概述了从结构角度了解到的关于该超家族不同进化枝中许多蛋白质在几种生物体中的细胞和生化功能。在人类中，DJ-1似乎主要作为一种氧化还原反应蛋白发挥作用，可能充当细胞氧化还原状态失衡的传感器。由于人类DJ-1的突变会导致某些类型的遗传性帕金森病，本章后面部分将重点讨论人类DJ-1中氧化翻译后修饰和致病突变的作用。
