DEFECTS IN MULLERIAN DUCT REGESSION

在狗的两种遗传性综合征中，苗勒管持续存在于 存在睾丸组织：XX性反转和持续性苗勒管 导管综合征（PMDS）。 本项目的目标是确定 在这些动物模型中， 人类中间性是由于缺乏穆勒抑制 物质（MIS）。 如果是这样，这些将是第一个定义的遗传缺陷， 目前还不太了解的MIS系统。 XX性逆转和PMDS犬将通过已知的育种方法生产 载波 将比较受影响幼崽性腺的MIS活性 与正常雄性同窝仔的性腺进行比较， MIS的培养测定。 MIS检测的结果将采用 关于性腺结构，特别是支持细胞的形态， 使用光学和电子显微镜。

In two hereditary syndromes of the dog, Mullerian ducts persist in the presence of testicular tissue: XX sex reversal and Persistent Mullerian Duct Syndrome (PMDS). The objective of this project is to determine whether the lack of Mullerian duct regression in these animal models of human intersexuality is the result of a deficiency in Mullerian Inhibiting Substance (MIS). If so, these will be the first defined genetic defects in the as yet poorly understood MIS system. Dogs with XX sex reversal and PMDS will be produced by breeding known carriers. The MIS activity of gonads from affected pups will be compared with that of gonads from normal male littermates using a graded organ culture assay for MIS. Results from MIS assays will be examined with respect to gonadal structure, particularly the morphology of Sertoli cells, using light and electron microscopy.