Persistent Müllerian Duct Syndrome

Anti-Mullerian hormone (AMH), also known as Mullerian-inhibiting substance, is produced by Sertoli cells of the fetal testis and normally causes regression of the Mullerian structures that would otherwise persist in the pathway of normal female development. AMH acts on anti-Mullerian hormone receptor, type II (AMHR2), causing regression of Mullerian ducts. In PMDS, failure to either secrete AMH or act on AMHR2 causes persistence of Mullerian structures in the male.