线粒体血红素代谢物：血红素合成和分布的复杂性研究进展。
The mitochondrial heme metabolon: Insights into the complex(ity) of heme synthesis and distribution.
机构信息
Department of Biochemistry and Molecular Biology, University of Georgia, Athens, GA 30602, United States.
Department of Biochemistry and Molecular Biology, University of Georgia, Athens, GA 30602, United States; Biomedical and Health Sciences Institute, University of Georgia, Athens, GA 30602, United States; Department of Microbiology, University of Georgia, Athens, GA, 30602, United States.
出版信息
Mol Genet Metab. 2019 Nov;128(3):198-203. doi: 10.1016/j.ymgme.2019.01.006. Epub 2019 Jan 17.
Heme is an essential cofactor in metazoans that is also toxic in its free state. Heme is synthesized by most metazoans and must be delivered to all cellular compartments for incorporation into a variety of hemoproteins. The heme biosynthesis enzymes have been proposed to exist in a metabolon, a protein complex consisting of interacting enzymes in a metabolic pathway. Metabolons enhance the function of enzymatic pathways by creating favorable microenvironments for pathway enzymes and intermediates, facilitating substrate transport, and providing a scaffold for interactions with other pathways, signaling molecules, or organelles. Herein we detail growing evidence for a mitochondrial heme metabolon and discuss its implications for the study of heme biosynthesis and cellular heme homeostasis.
血红素是真核生物必需的辅因子，但在游离状态下也是有毒的。血红素由大多数真核生物合成，必须递送到所有细胞区室以掺入各种血红素蛋白中。血红素生物合成酶已被提议存在于代谢物中，这是一种由代谢途径中相互作用的酶组成的蛋白质复合物。代谢物通过为途径酶和中间产物创造有利的微环境、促进底物运输以及为与其他途径、信号分子或细胞器的相互作用提供支架，来增强酶促途径的功能。本文详细介绍了线粒体血红素代谢物的日益增多的证据，并讨论了其对血红素生物合成和细胞血红素动态平衡研究的意义。
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