MD, PhD Micheline Misrahi (Professor of Biochemistry and Molecular Biology) , PhD Isabelle Beau (Assistant Lecturer) , MD Geri Meduri (Attaché) , MD Claire Bouvattier (Chief Resident) , PhD Michel Atger (Researcher) , PhD Hughes Loosfelt (Researcher) , PhD Nicoleas Ghinea (Researcher) , PhD Mai Vu Hai (Researcher) , MD, PhD Pierre F. Bougnères (Professor of Paediatrics) , MD, DSc Edwin Milgrom (Professor of Biochemistry and Molecular Biology)
{"title":"Gonadotropin receptors and the control of gonadal steroidogenesis: Physiology and pathology","authors":"MD, PhD Micheline Misrahi (Professor of Biochemistry and Molecular Biology) , PhD Isabelle Beau (Assistant Lecturer) , MD Geri Meduri (Attaché) , MD Claire Bouvattier (Chief Resident) , PhD Michel Atger (Researcher) , PhD Hughes Loosfelt (Researcher) , PhD Nicoleas Ghinea (Researcher) , PhD Mai Vu Hai (Researcher) , MD, PhD Pierre F. Bougnères (Professor of Paediatrics) , MD, DSc Edwin Milgrom (Professor of Biochemistry and Molecular Biology)","doi":"10.1016/S0950-351X(98)80444-8","DOIUrl":null,"url":null,"abstract":"<div><p>Over the past few years, knowledge of the structure of gonadotropin receptors and their mode of action has rapidly advanced. The cDNA corresponding to the luteinizeng hormone (LH) receptor (LHR) has been cloned, leading to the identification of a novel family of G-protein-coupled receptors. The follicle stimulating hormone (FSH) receptor (FSHR) was thereafter cloned by cross-hybridization with the LHR. Structure—function relationships have been studied by mutagenesis experiments in several laboratories. The cloning and chromosomal localization to chromosome 2p21 of the two human gonadotropin receptor genes has provided insights into their evolutionary relationships. The LHR and FSHR genes are very large and contain 10 and 11 exons respectively.</p><p>The obtention of monoclonal antibodies against the receptors resulted in the characterization of the receptor proteins. These antibodies also allowed the study of receptor expression in target cells in physiological and pathological conditions. The internalization of the LHR has been studied by electron microscopy. A mechanism of receptor-mediated transcytosis through the endothelial cells of the testes has been described for the LHR. The polarized expression of receptors has been studied.</p><p>The cloning of gonadotropin receptor genes has opened the field of genetic study of the receptors. Inactivating mutations of the LHR have been described in Leydig cell agenesis or hypoplasia. Different phenotypes, including complete pseudohermaphroditism, ambiguous genitalia and male phenotype, have been described. In the case of the FSHR, only one mutation has been reported in familial ovarian dysgenesis with primary amenorrhea. Related males have variable alterations of spermatogenesis and fertility. Constitutive mutations of the LHR have been reported in familial testotoxicosis. One similar mutation has also been described for the FSHR. Such mutations may lead to the development of a model of receptor activation.</p></div>","PeriodicalId":77027,"journal":{"name":"Bailliere's clinical endocrinology and metabolism","volume":"12 1","pages":"Pages 35-66"},"PeriodicalIF":0.0000,"publicationDate":"1998-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0950-351X(98)80444-8","citationCount":"42","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Bailliere's clinical endocrinology and metabolism","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0950351X98804448","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 42
Abstract
Over the past few years, knowledge of the structure of gonadotropin receptors and their mode of action has rapidly advanced. The cDNA corresponding to the luteinizeng hormone (LH) receptor (LHR) has been cloned, leading to the identification of a novel family of G-protein-coupled receptors. The follicle stimulating hormone (FSH) receptor (FSHR) was thereafter cloned by cross-hybridization with the LHR. Structure—function relationships have been studied by mutagenesis experiments in several laboratories. The cloning and chromosomal localization to chromosome 2p21 of the two human gonadotropin receptor genes has provided insights into their evolutionary relationships. The LHR and FSHR genes are very large and contain 10 and 11 exons respectively.
The obtention of monoclonal antibodies against the receptors resulted in the characterization of the receptor proteins. These antibodies also allowed the study of receptor expression in target cells in physiological and pathological conditions. The internalization of the LHR has been studied by electron microscopy. A mechanism of receptor-mediated transcytosis through the endothelial cells of the testes has been described for the LHR. The polarized expression of receptors has been studied.
The cloning of gonadotropin receptor genes has opened the field of genetic study of the receptors. Inactivating mutations of the LHR have been described in Leydig cell agenesis or hypoplasia. Different phenotypes, including complete pseudohermaphroditism, ambiguous genitalia and male phenotype, have been described. In the case of the FSHR, only one mutation has been reported in familial ovarian dysgenesis with primary amenorrhea. Related males have variable alterations of spermatogenesis and fertility. Constitutive mutations of the LHR have been reported in familial testotoxicosis. One similar mutation has also been described for the FSHR. Such mutations may lead to the development of a model of receptor activation.
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