As mentioned above, Type 2c VHL disease has perplexed the field as Type 2c pVHL mutants appear to regulate HIFα in a manner similar to wild-type pVHL, which would suggest that pheochromocytoma is uncoupled from HIFα degradation (Hoffman et al., 2001; Clifford et al., 2001). Here, VHL is linked to von Hippel-Lindau disease.