Despite Type 1 disease driven by mutations predicted to profoundly inactivate pVHL, including nonsense, frameshift and splicing mutations, these patients do not develop pheochromocytoma (Stebbins et al., 1999; Chen et al., 1995; Crossey et al., 1995; Stolle et al., 1998). The gene discussed is VHL; the disease is hereditary pheochromocytoma-paraganglioma.