Dominiak Karolina, Jarmuszkiewicz Wieslawa
Zakład Bioenergetyki, Instytut Biologii Molekularnej i Biotechnologii, Uniwersytet im. Adama Mickiewicza w Poznaniu.
Postepy Biochem. 2020 Jan 8;65(4):271-277. doi: 10.18388/pb.2019_289.
Coenzyme Q is a fat-soluble molecule present in all cell membranes, including the inner mitochondrial membrane. Mitochondrial Q (mQ) is a key electron carrier in the respiratory chain and an important antioxidant. On the other hand, mQ participates in the production by respiratory chain of mitochondrial reactive oxygen species (mROS) that are formed as a byproduct of oxygen metabolism or under oxidative stress conditions. Increased mROS production can lead to a series of oxidative damage that underlies cell aging or a number of diseases. In addition, mROS act as signaling molecules. Respiratory chain electron carriers, primarily mQ-related protein complexes, are considered the main mROS production sites. With age, the level of Q, and in particular its reduced form, decreases in the body. Disorders associated with coenzyme Q deficiency are mainly associated with excessive mROS production and a decrease in ATP production, which may result in mitochondrial, cardiovascular or neurodegenerative diseases.
辅酶Q是一种存在于所有细胞膜（包括线粒体内膜）中的脂溶性分子。线粒体Q（mQ）是呼吸链中的关键电子载体和重要的抗氧化剂。另一方面，mQ参与呼吸链产生线粒体活性氧（mROS），mROS是氧代谢的副产物或在氧化应激条件下形成的。mROS产生增加会导致一系列氧化损伤，这是细胞衰老或许多疾病的基础。此外，mROS作为信号分子发挥作用。呼吸链电子载体，主要是与mQ相关的蛋白质复合物，被认为是主要的mROS产生部位。随着年龄的增长，体内Q的水平，尤其是其还原形式，会下降。与辅酶Q缺乏相关的疾病主要与mROS产生过多和ATP产生减少有关，这可能导致线粒体、心血管或神经退行性疾病。
