Prohormone convertase 1 (PCSK1) is an enzyme involved in the cleavage of POMC into ACTH, which is then further cleaved to make α-MSH by carboxypeptidase E. Humans lacking PCSK1 are severely obese and have glucocorticoid deficiency, hypogonadotropic hypogonadism, and postprandial hypoglycemia, which occurs as a result of impaired processing of proinsulin to insulin by PCSK1 (Farooqi et al. 2007b). The gene discussed is PCSK1; the disease is hypogonadotropic hypogonadism.