Luteinizing hormone-releasing hormone (LHRH)-expressing cells do not migrate normally in an inherited hypogonadal (Kallmann) syndrome.
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.
Schwanzel-Fukuda
M
M
Rockefeller University, Laboratory of Neurobiology and Behavior, New York, NY 10021.
Bick
D
D
Pfaff
D W
DW
D001921:Brain / Q000196:embryology / Q000378:metabolism / Q000473:pathology*; D002869:Chromosome Aberrations; D025063:Chromosome Disorders; D005333:Fetus; D005865:Gestational Age; D007987:Gonadotropin-Releasing Hormone / Q000378:metabolism*; D006801:Humans; D007006:Hypogonadism / Q000235:genetics* / Q000378:metabolism; D008297:Male; D000857:Olfaction Disorders / Q000150:complications* / Q000235:genetics; D013577:Syndrome; D014960:X Chromosome
D007987:Gonadotropin-Releasing Hormone