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Complex II of the respiratory chain, which is specifically involved in the oxidation of succinate, carries electrons from FADH to CoQ. The complex is composed of four nuclear-encoded subunits and is localized in the mitochondrial inner membrane. The iron-sulfur subunit is highly conserved and contains three cysteine-rich clusters which may comprise the iron-sulfur centers of the enzyme. Sporadic and familial mutations in this gene result in paragangliomas and pheochromocytoma, and support a link between mitochondrial dysfunction and tumorigenesis.
Gene Name: | succinate dehydrogenase complex, subunit B, iron sulfur (Ip) |
Synonyms: | SDHB, SDH1, SDHIP, SDH, IP, PGL4, SDH2 |
Target Sequences: | NM_003000 NP_002991.2 P21912 |
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