Mutations in SDHA, SDHB, SDHC, SDHD and SDH assembly factor 2 (SDHAF2) have been identified in hereditary paragangliomas (hPGLs) and pheochromocytomas (PCCs) (Table 1) (Baysal et al., 2000; Niemann and Muller, 2000; Astuti et al., 2001; Baysal et al., 2002; Hao et al., 2009; Bayley et al., 2010; Burnichon et al., 2010). Here, SDHAF2 is linked to hereditary pheochromocytoma-paraganglioma.