title: Perspectives in Pediatric Pathology, Chapter 6. Male Undermasculinization.
abstract: Normal male development requires three conditions: (1) adequate differentiation of the fetal testis; (2) synthesis and secretion of testicular hormones; and (3) effective action of these hormones on target organs. This requires the combined action of the inhibitory anti-müllerian hormone (AMH, secreted by Sertoli cells) to block the development of the uterus and fallopian tubes from the müllerian duct, together with the trophic stimulus of testosterone (a Leydig cell product), which leads to virilization of the wolffian ducts. Additionally, the development of external genitalia depends on the conversion of testosterone to dihydrotestosterone by the enzyme 5-α-reductase. Failure of any of these mechanisms leads to deficient virilization or the so-called "male pseudohermaphroditism" syndromes.
authors.affiliation: 1 Pathology, Hospital La Paz, Universidad Autónoma de Madrid, Calle Arzobispo Morcillo #2, Madrid 28029, Spain.
authors.forename: Manuel
authors.initials: M
authors.lastname: Nistal
authors.affiliation: 2 Department of Cell Biology, Universidad de Alcala, Madrid, Spain.
authors.forename: Ricardo
authors.initials: R
authors.lastname: Paniagua
authors.affiliation: 1 Pathology, Hospital La Paz, Universidad Autónoma de Madrid, Calle Arzobispo Morcillo #2, Madrid 28029, Spain.
authors.forename: Pilar
authors.initials: P
authors.lastname: González-Peramato
authors.affiliation: 3 Department of Pathology, Children's Hospital of Pittsburgh of UPMC, One Children's Hospital Drive, 4401 Penn Avenue, Pittsburgh, PA 15224, USA.
authors.forename: Miguel
authors.initials: M
authors.lastname: Reyes-Múgica
mesh_terms: D013741:3-Oxo-5-alpha-Steroid 4-Dehydrogenase / Q000378:metabolism; D054304:Anti-Mullerian Hormone / Q000378:metabolism; D001706:Biopsy; D002454:Cell Differentiation; D012734:Disorders of Sex Development / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D008297:Male; D057228:Masculinity; D010641:Phenotype; D011237:Predictive Value of Tests; D019849:Sex Determination Processes; D015398:Signal Transduction; D013737:Testis / Q000378:metabolism / Q000473:pathology*; D013739:Testosterone / Q000378:metabolism; D014770:Virilism
chemical_list: D013739:Testosterone; D054304:Anti-Mullerian Hormone; D013741:3-Oxo-5-alpha-Steroid 4-Dehydrogenase