Komada and co-workers demonstrated impairment of insulin secretion, particularly in an early phase of the insulin secretory response in 13 patients with phaeochromocytoma/paraganglioma (extra-adrenal phaeochromocytoma) (11 with phaeochromocytoma and two with paraganglioma) (32). The gene discussed is INS; the disease is pheochromocytoma.