The identification, characterization, and mutational analysis of three
different genes. namely the prepro-arginine-vasopressin-neurophysin I
I gene (prepro-AVP-NPII), the arginine-vasopressin receptor 2 gene (AV
PR2), and the vasopressin-sensitive water channel gene (aquaporin-2, A
QP2), provide the basis for our understanding of three different hered
itary forms of diabetes insipidus: autosomal dominant neurogenic diabe
tes insipidus, X-linked nephrogenic diabetes insipidus, and autosomal
recessive nephrogenic diabetes insipidus, respectively. These advances
provide diagnostic tools for physicians caring for these patients.