Succinate dehydrogenase, also known as Complex II or succinate:quinone oxidoreductase, is a key component of the citric acid cycle and the electron transport chain. Specifically, it is involved in the oxidation of succinate. SDH consists of four subunits: SDHA, SDHB, SDHC, and SDHD. Research studies have shown that defects in SDHA cause complex II deficiency. In addition, investigators have observed reduction of SDHA in the striatum of patients with Huntington’s disease, and reduction of SDHB, SDHC, and SDHD in paragangliomas and phenochromocytomas.
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