Endocrine Abstracts

Objectives: Male pseudohermaphroditism, or Leydig cell hypoplasia (LCH), is an autosomal recessive disorder in individuals with a 46, XY karyotype, characterized by a predominantly female phenotype despite the presence of testicular structures. It is caused by mutations in the luteinizing hormone/chorionic gonadotropin receptor gene (LHCGR), which impair either LH/CG binding or signal transduction. However, molecular analysis has revealed that the LHCGR is apparently normal in...

Male sexual development and masculinization are triggered by two hormones, CG and LH. While CG, produced by the trophoblasts during pregnancy, is essential for normal development of male secondary sex organs, pituitary LH is indispensable for virilization and initiation and maintenance of spermatogenesis. Both hormones exert their action via the LH receptor (LHR) expressed in the Leydig cells by stimulating testosterone biosynthesis. This two hormone/one receptor system is exc...