Ultrastructural analysis of the testes in male pseudohermaphrodism reductase.
Light and electron microscopic observations of the testes in male pseudohermaphrodism due to deficiency of 17-ketosteroid reductase demonstrate an increased thickness of the walls of the seminiferous tubules. Although the seminiferous tubules are filled mostly by Sertoli cells containing crystalloids of Charcot-Bottcher, clusters of spermatogonia and spermatocytes are located at infrequent intervals along their lengths. Differentiation of spermatogenetic cells beyond the spermatocyte stage was not observed. Hyperplasia of Leydig cells, which are structurally similar to those of the normal testis, was pronounced. Pigment bodies were present in Leydig cells, whereas crystals of Reinke were not observed. The decreases in plasma androstenedione, testosterone and estrone following orchiectomy and the presence of a well-developed system of organelles (smooth endoplasmic reticulum and mitochondria containing tubular cristae), typical of steroid-secreting cells, indicate that the Leydig cells were active in steroid hormone synthesis, albeit deficient in 17-ketosteroid reductase activity.
D015068:17-Ketosteroids / Q000378:metabolism*; D002466:Cell Nucleolus / Q000648:ultrastructure; D002467:Cell Nucleus / Q000648:ultrastructure; D012734:Disorders of Sex Development / Q000201:enzymology / Q000378:metabolism / Q000473:pathology*; D004721:Endoplasmic Reticulum / Q000648:ultrastructure; D006056:Golgi Apparatus / Q000648:ultrastructure; D006801:Humans; D002479:Inclusion Bodies / Q000648:ultrastructure; D007365:Intercellular Junctions / Q000648:ultrastructure; D007985:Leydig Cells / Q000378:metabolism / Q000648:ultrastructure; D008297:Male; D008928:Mitochondria / Q000648:ultrastructure; D009940:Organoids / Q000648:ultrastructure; D010088:Oxidoreductases / Q000172:deficiency* / Q000378:metabolism; D012671:Seminiferous Tubules / Q000648:ultrastructure; D012708:Sertoli Cells / Q000648:ultrastructure; D013090:Spermatocytes / Q000648:ultrastructure; D013093:Spermatogonia / Q000648:ultrastructure; D013256:Steroids / Q000096:biosynthesis; D013737:Testis / Q000648:ultrastructure*
D015068:17-Ketosteroids; D013256:Steroids; D010088:Oxidoreductases