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Indexed/Abstracted in: EMBASE, PubMed/MEDLINE, Science Citation Index Expanded (SciSearch), Scopus
Impact Factor 1,118
Online ISSN 1827-1634
GnRH NEURON BIOLOGY AND CONGENITAL HYPOGONADOTROPIC HYPOGONADISM
Ivan BASSI 1, 2, Valentina ANDRÉ 3, Federica MARELLI 1, Valeria VEZZOLI 4, Giorgio R. MERLO 5, Anna CARIBONI 3, Luca PERSANI 1, 4, Yoav GOTHILF 6, Marco BONOMI 1, 4
1 Division of Endocrine and Metabolic Diseases & Lab. of Endocrine and Metabolic Research, IRCCS Istituto Auxologico Italiano, Milan, Italy; 2 Department of Health Science, University of Milan, Milan, Italy; 3 Department of Pharmacological and Biomolecular Sciences, University of Milan, Milan, Italy; 4 Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy; 5 Department of Molecular Biotechnology and Health Sciences, University of Turin, Turin, Italy; 6 Department of Neurobiology, The George S. Wise Faculty of Life Sciences and Sagol School of Neuroscience, Tel-Aviv University, Tel-Aviv, Israel
Gonadotropin-releasing hormone (GnRH) neurons have a pivotal role in the physiological functions of hypotahlamic-pituitary-gonadal (HPG) axis. The pulsatile releasing of GnRH hormone into the hypophyseal portal circulation at the median eminence represent the first domino in the HPG cascade of events that regulate the development, fertility and aging in all vertebrates. These neurons principally originate in the olfactory placode and migrate during early embryonal stages into the hypothalamus. Alterations in developmental processes or in the releasing of GnRH hormone lead to a rare and complex disorder of the reproductive axis called congenital hypogonadotropic hypogonadism (CHH). Genetic screening of human patients and the use of model systems have led to the identification of several genes involved in the CHH pathogenesis underlying its oligogenic nature. Nevertheless CHH remains, for a large cohort of patients, idiopathic and GnRH neurogenesis processes not fully understood. This is due to intrinsic difficulties that exist in the analysis of earliest embryonic developmental stages and in the methodologies developed to study the CHH-causing genes. In this regard, zebrafish embryos, on account of its external fertilization and development, allow a real-time analysis that could overcome some of the above mentioned limitations. Moreover, the recent availability of several transgenic zebrafish reporter lines makes it an excellent model for the study of the oligogenic mechanisms leading to CHH.