EPAS1 and hereditary pheochromocytoma-paraganglioma: However, recent developments have changed this: The first successful pseudohypoxic PPGL GEMM was recently developed with a gain-of-function EPAS1 mutation, characterizing Pacak-Zhuang syndrome [148], and tetracycline-induced dual SDHB/NF1 mouse model was recently developed (showing that SDHB inactivation alone was insufficient for tumorigenesis, but coupling with NF1 lead to pheochromocytoma) [91,151].