Schwanzel-Fukuda M, Bick D, Pfaff D W
Rockefeller University, Laboratory of Neurobiology and Behavior, New York, NY 10021.
Brain Res Mol Brain Res. 1989 Dec;6(4):311-26. doi: 10.1016/0169-328x(89)90076-4.
Kallmann syndrome inherited hypogonadotropic hypogonadism with anosmia, is associated with an X-chromosome deletion at Xp 22.3. In a Kallmann fetus, we have found an absence of luteinizing hormone-releasing hormone (LHRH)-expressing cells in the brain despite dense clusters of LHRH cells and fibers in the nose. LHRH-containing cells and neurites end in a tangle beneath the forebrain, within the dural layers of the meninges, on the dorsal surface of the cribriform plate of the ethmoid bone. Normal fetal brains, matched for age and sex, had LHRH cells and fibers, as expected, in the hypothalamus and preoptic area. Since LHRH-expressing cells recently were discovered to migrate from the olfactory placode into the brain, it appears that the hypogonadotropism of the Kallmann syndrome can be accounted for by a failure of LHRH cells to migrate into the brain.
卡尔曼综合征是一种遗传性低促性腺激素性性腺功能减退伴嗅觉缺失的疾病，与X染色体Xp 22.3区域的缺失有关。在一个卡尔曼综合征胎儿中，我们发现其大脑中缺乏表达促黄体生成素释放激素（LHRH）的细胞，尽管在鼻子中有密集的LHRH细胞和纤维簇。含有LHRH的细胞和神经突在前脑下方、脑膜的硬脑膜层内、筛骨筛板的背表面处缠结终止。与之年龄和性别匹配的正常胎儿大脑，如预期的那样，在下丘脑和视前区有LHRH细胞和纤维。由于最近发现表达LHRH的细胞从嗅基板迁移到大脑中，因此卡尔曼综合征的低促性腺激素状态似乎可以用LHRH细胞未能迁移到大脑中来解释。
