Follicle-stimulating hormone receptor (FSH-R) is the receptor for follicle-stimulating hormone (FSH). The activity of this receptor is mediated by G proteins which activate adenylate cyclase. Defects in FSH-R are a cause of ovarian dysgenesis type 1 (ODG1), also known as premature ovarian failure, gonadal dysgenesis , Xenopus/Amphibian,, Xenopus/Amphibian, type, , Xenopus/Amphibian,, Xenopus/Amphibian, gonadal dysgenesis (, Xenopus/Amphibian,, Xenopus/Amphibian,GD), hereditary hypergonadotropic ovarian failure, or hypergonadotropic ovarian dysgenesis with normal karyotype. ODG1 is an autosomal recessive disease characterized by primary amenorrhea, variable development of secondary se, Xenopus/Amphibian, characteristics, and high serum levels of follicle-stimulating hormone (FSH) and luteinizing hormone (LH).