title: GnRH, anosmia and hypogonadotropic hypogonadism--where are we?
abstract: Gonadotropin releasing hormone (GnRH) neurons originate the nasal placode and migrate into the brain during prenatal development. Once within the brain, these cells become integral components of the hypothalamic-pituitary-gonadal axis, essential for reproductive function. Disruption of this system causes hypogonadotropic hypogonadism (HH). HH associated with anosmia is clinically defined as Kallman syndrome (KS). Recent work examining the developing nasal region has shed new light on cellular composition, cell interactions and molecular cues responsible for the development of this system in different species. This review discusses some developmental aspects, animal models and current advancements in our understanding of pathologies affecting GnRH. In addition we discuss how development of neural crest derivatives such as the glia of the olfactory system and craniofacial structures control GnRH development and reproductive function.
authors.affiliation: Department of Biological Sciences and the Center for Neuroscience Research, University at Albany, State University of New York, Albany, NY 12222, United States. Electronic address: pforni@albany.edu.
authors.forename: Paolo E
authors.initials: PE
authors.lastname: Forni
authors.affiliation: Cellular and Developmental Neurobiology Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, United States. Electronic address: wrays@ninds.nih.gov.
authors.forename: Susan
authors.initials: S
authors.lastname: Wray
mesh_terms: D000818:Animals; D001921:Brain / Q000378:metabolism*; D007987:Gonadotropin-Releasing Hormone / Q000378:metabolism*; D007006:Hypogonadism / Q000378:metabolism; D017436:Kallmann Syndrome / Q000378:metabolism*; D009474:Neurons / Q000378:metabolism*; D000857:Olfaction Disorders / Q000378:metabolism
chemical_list: D007987:Gonadotropin-Releasing Hormone
keywords: Craniofacial defects; GnRH; Hypogonadotropic hypogonadism; Hypothalamic–pituitary–gonadal (HPG) axis; Kallmann syndrome; Neural crest; Olfactory ensheathing cells; Olfactory placode; Waardenburg syndrome