Van Vranken Jonathan G, Na Un, Winge Dennis R, Rutter Jared
Department of Biochemistry and.
Crit Rev Biochem Mol Biol. 2015 Mar-Apr;50(2):168-80. doi: 10.3109/10409238.2014.990556. Epub 2014 Dec 9.
Succinate dehydrogenase (or complex II; SDH) is a heterotetrameric protein complex that links the tribarboxylic acid cycle with the electron transport chain. SDH is composed of four nuclear-encoded subunits that must translocate independently to the mitochondria and assemble into a mature protein complex embedded in the inner mitochondrial membrane. Recently, it has become clear that failure to assemble functional SDH complexes can result in cancer and neurodegenerative syndromes. The effort to thoroughly elucidate the SDH assembly pathway has resulted in the discovery of four subunit-specific assembly factors that aid in the maturation of individual subunits and support the assembly of the intact complex. This review will focus on these assembly factors and assess the contribution of each factor to the assembly of SDH. Finally, we propose a model of the SDH assembly pathway that incorporates all extant data.
琥珀酸脱氢酶（或复合体II；SDH）是一种异源四聚体蛋白复合体，它将三羧酸循环与电子传递链连接起来。SDH由四个核编码亚基组成，这些亚基必须独立转运至线粒体，并组装成嵌入线粒体内膜的成熟蛋白复合体。最近，已明确无法组装功能性SDH复合体可导致癌症和神经退行性综合征。为彻底阐明SDH组装途径所做的努力已促成发现了四种亚基特异性组装因子，这些因子有助于单个亚基的成熟并支持完整复合体的组装。本综述将聚焦于这些组装因子，并评估每个因子对SDH组装的贡献。最后，我们提出了一个整合所有现有数据的SDH组装途径模型。
