Succinate Dehydrogenase Complex Assembly Factor 2 (SDHAF2) is a crucial protein involved in the assembly and function of the succinate dehydrogenase (SDH) enzyme complex, also known as respiratory complex II. This enzyme complex plays a dual role in cellular respiration by participating in both the tricarboxylic acid (TCA) cycle and the mitochondrial electron transport chain (ETC) .
The SDHAF2 gene provides instructions for making a protein that interacts with the SDH enzyme. The SDHAF2 protein helps a molecule called flavin adenine dinucleotide (FAD) attach to the SDH enzyme. FAD is a cofactor that is essential for the enzyme’s activity . The SDH enzyme complex consists of multiple subunits and requires a series of assembly factors, including SDHAF2, for its proper assembly and function .
SDH, also known as succinate:ubiquinone oxidoreductase, is unique in that it is involved in both the TCA cycle and the ETC. In the TCA cycle, SDH catalyzes the oxidation of succinate to fumarate. In the ETC, it transfers electrons from succinate to ubiquinone, contributing to the generation of ATP through oxidative phosphorylation .
Dysfunction of the SDH enzyme complex, including defects in SDHAF2, has been linked to a variety of human diseases. These include certain types of cancer and neurodegenerative disorders. The proper assembly and function of SDH are critical for cellular energy production, and defects in this process can lead to significant metabolic disturbances .