المؤلفون: Relav, Lauriane, Doghman-Bouguerra, Mabrouka, Ruggiero, Carmen, Muzzi, João, C D, Figueiredo, Bonald, C, Lalli, Enzo

المساهمون: Institut de pharmacologie moléculaire et cellulaire (IPMC), Université Nice Sophia Antipolis (1965 - 2019) (UNS)-Centre National de la Recherche Scientifique (CNRS)-Université Côte d'Azur (UniCA), Instituto de Pesquisa Pelé Pequeno Principe, ANR-20-CE14-0007,Goldilocks,Analyse intégrée du rôle du facteur de transcription SF-1 / NR5A1 et de ses gènes cibles dépendants du dosage dans la fonction gonadique et les troubles du développement sexuel (DSD)(2020)

المصدر: ISSN: 1661-6596.

مصطلحات موضوعية: Steroidogenic Factor 1, transcription factors, nuclear receptors, adrenal cortex, steroidogenesis, gene dosage, tumorigenesis, adrenocortical carcinoma, [SDV]Life Sciences [q-bio]

الوصف: International audience ; Steroidogenic factor-1 (SF-1, also termed Ad4BP; NR5A1 in the official nomenclature) is a nuclear receptor transcription factor that plays a crucial role in the regulation of adrenal and gonadal development, function and maintenance. In addition to its classical role in regulating the expression of P450 steroid hydroxylases and other steroidogenic genes, involvement in other key processes such as cell survival/proliferation and cytoskeleton dynamics have also been highlighted for SF-1. SF-1 has a restricted pattern of expression, being expressed along the hypothalamic-pituitary axis and in steroidogenic organs since the time of their establishment. Reduced SF-1 expression affects proper gonadal and adrenal organogenesis and function. On the other hand, SF-1 overexpression is found in adrenocortical carcinoma and represents a prognostic marker for patients’ survival. This review is focused on the current knowledge about SF-1 and the crucial importance of its dosage for adrenal gland development and function, from its involvement in adrenal cortex formation to tumorigenesis. Overall, data converge towards SF-1 being a key player in the complex network of transcriptional regulation within the adrenal gland in a dosage-dependent manner.

العلاقة: hal-04195897; https://hal.science/hal-04195897Test; https://hal.science/hal-04195897/documentTest; https://hal.science/hal-04195897/file/IJMS%202023.pdfTest

الإتاحة: https://doi.org/10.3390/ijms24043585Test
https://hal.science/hal-04195897Test
https://hal.science/hal-04195897/documentTest
https://hal.science/hal-04195897/file/IJMS%202023.pdfTest

المؤلفون: Olga Glazova, Asya Bastrich, Andrei Deviatkin, Nikita Onyanov, Samira Kaziakhmedova, Liudmila Shevkova, Nawar Sakr, Daria Petrova, Maria V. Vorontsova, Pavel Volchkov

المصدر: International Journal of Molecular Sciences; Volume 24; Issue 6; Pages: 5365

مصطلحات موضوعية: adrenal cortex, cell differentiation, steroidogenic factor 1, CAH, organoids, murine models, single cell RNA sequencing

جغرافية الموضوع: agris

الوصف: The adrenal glands are important endocrine organs that play a major role in the stress response. Some adrenal glands abnormalities are treated with hormone replacement therapy, which does not address physiological requirements. Modern technologies make it possible to develop gene therapy drugs that can completely cure diseases caused by mutations in specific genes. Congenital adrenal hyperplasia (CAH) is an example of such a potentially treatable monogenic disease. CAH is an autosomal recessive inherited disease with an overall incidence of 1:9500–1:20,000 newborns. To date, there are several promising drugs for CAH gene therapy. At the same time, it remains unclear how new approaches can be tested, as there are no models for this disease. The present review focuses on modern models for inherited adrenal gland insufficiency and their detailed characterization. In addition, the advantages and disadvantages of various pathological models are discussed, and ways of further development are suggested.

وصف الملف: application/pdf

العلاقة: Molecular Pathology, Diagnostics, and Therapeutics; https://dx.doi.org/10.3390/ijms24065365Test

الإتاحة: https://doi.org/10.3390/ijms24065365Test

المؤلفون: Lauriane Relav, Mabrouka Doghman-Bouguerra, Carmen Ruggiero, João C. D. Muzzi, Bonald C. Figueiredo, Enzo Lalli

المصدر: International Journal of Molecular Sciences; Volume 24; Issue 4; Pages: 3585

مصطلحات موضوعية: steroidogenic factor 1, transcription factors, nuclear receptors, adrenal cortex, steroidogenesis, gene dosage, tumorigenesis, adrenocortical carcinoma

جغرافية الموضوع: agris

الوصف: Steroidogenic factor-1 (SF-1, also termed Ad4BP; NR5A1 in the official nomenclature) is a nuclear receptor transcription factor that plays a crucial role in the regulation of adrenal and gonadal development, function and maintenance. In addition to its classical role in regulating the expression of P450 steroid hydroxylases and other steroidogenic genes, involvement in other key processes such as cell survival/proliferation and cytoskeleton dynamics have also been highlighted for SF-1. SF-1 has a restricted pattern of expression, being expressed along the hypothalamic-pituitary axis and in steroidogenic organs since the time of their establishment. Reduced SF-1 expression affects proper gonadal and adrenal organogenesis and function. On the other hand, SF-1 overexpression is found in adrenocortical carcinoma and represents a prognostic marker for patients’ survival. This review is focused on the current knowledge about SF-1 and the crucial importance of its dosage for adrenal gland development and function, from its involvement in adrenal cortex formation to tumorigenesis. Overall, data converge towards SF-1 being a key player in the complex network of transcriptional regulation within the adrenal gland in a dosage-dependent manner.

وصف الملف: application/pdf

العلاقة: Molecular Oncology; https://dx.doi.org/10.3390/ijms24043585Test

الإتاحة: https://doi.org/10.3390/ijms24043585Test

المؤلفون: Xu, B, Yang, WH, Gerin, I, Hu, CD, Hammer, GD, Koenig, RJ

مصطلحات موضوعية: Adrenal Cortex, Amino Acid Sequence, Amino Acid Substitution, Animals, Cell Line, DAX-1 Orphan Nuclear Receptor, DNA-Binding Proteins, Humans, Intracellular Space, Male, Mice, Molecular Sequence Data, Mutant Proteins, Nuclear Receptor Coactivator 2, Phosphoproteins, Promoter Regions, Genetic, Protein Binding, Protein Transport, RNA, Long Noncoding, Small Interfering, Untranslated, Receptors, Retinoic Acid, Repressor Proteins, Steroidogenic Factor 1, Steroids, Testis, Trans-Activators

جغرافية الموضوع: Qingdao, China

الوصف: The nuclear receptor steroidogenic factor 1 (SF-1) is essential for adrenal development and steroidogenesis. The atypical orphan nuclear receptor Dax-1 binds to SF-1 and represses SF-1 target genes. Paradoxically, however, loss-of-function mutations of Dax-1 also cause adrenal hypoplasia, suggesting that Dax-1 may function as an SF-1 coactivator under some circumstances. Indeed, we found that Dax-1 can function as a dosage-dependent SF-1 coactivator. Both SF-1 and Dax-1 bind to steroid receptor RNA activator (SRA), a coactivator that functions as an RNA. The coactivator TIF2 also associates with Dax-1 and synergistically coactivates SF-1 target gene transcription. A naturally occurring Dax-1 mutation inhibits this transactivation, and the mutant Dax-1-TIF2 complex mislocalizes in living cells. Coactivation by Dax-1 is abolished by SRA knockdown. The expression of the steroidogenic gene products steroidogenic acute regulatory protein (StAR) and melanocortin 2 receptor is reduced in adrenal Yl cells following the knockdown of endogenous SRA. Similarly, the knockdown of endogenous Dax-1 downregulates the expression of the steroidogenic gene products CYP11A1 and StAR in both H295R adrenal and MA-10 Leydig cells. These findings reveal novel functions of SRA and Dax-1 in steroidogenesis and adrenal biology. Copyright © 2009, American Society for Microbiology. ; Experimental Marine Biological Laboratory, Institute of Oceanology, Academia Sinica ; http://deepblue.lib.umich.edu/bitstream/2027.42/191162/2/XuTest et al., Dax-1 JCB 2009 1010-08.pdf ; Description of Xu et al., Dax-1 JCB 2009 1010-08.pdf : Published version

وصف الملف: application/pdf

العلاقة: https://www.ncbi.nlm.nih.gov/pubmed/19188450Test; https://hdl.handle.net/2027.42/191162Test; https://dx.doi.org/10.7302/21551Test; orcid:0000-0001-7429-3908; orcid:0000-0001-6843-3628; orcid:0000-0003-0021-4976; Xu, B; 0000-0001-7429-3908; Yang, WH; Gerin, I; Hu, CD; Hammer, GD; 0000-0001-6843-3628; Koenig, RJ; 0000-0003-0021-4976

الإتاحة: https://doi.org/10.1128/MCB.01010-08Test
https://doi.org/10.7302/21551Test
https://hdl.handle.net/2027.42/191162Test
https://www.ncbi.nlm.nih.gov/pubmed/19188450Test

المؤلفون: Ruiz-Babot, G., Balyura, M., Hadjidemetriou, I., Ajodha, S.J., Taylor, D.R., Ghataore, L., Taylor, N.F., Schubert, U., Ziegler, C.G., Storr, H.L., Druce, M.R., Gevers, E.F., Drake, W.M., Srirangalingam, U., Conway, G.S., King, P.J., Metherell, L.A., Bornstein, S.R., Guasti, L.

المصدر: Cell Rep. 22, 1236-1249 (2018)

مصطلحات موضوعية: Nr5a1, Adrenal Cortex, Adrenal Insufficiency, Congenital Adrenal Hyperplasia, Disease Modeling, Reprogramming, Steroidogenic Cells, Steroidogenic Factor 1, Transplantation, Urine-derived Stem Cells

الوصف: Adrenal insufficiency is managed by hormone replacement therapy, which is far from optimal; the ability to generate functional steroidogenic cells would offer a unique opportunity for a curative approach to restoring the complex feedback regulation of the hypothalamic-pituitary-adrenal axis. Here, we generated human induced steroidogenic cells (hiSCs) from fibroblasts, blood-, and urine-derived cells through forced expression of steroidogenic factor-1 and activation of the PKA and LHRH pathways. hiSCs had ultrastructural features resembling steroid-secreting cells, expressed steroidogenic enzymes, and secreted steroid hormones in response to stimuli. hiSCs were viable when transplanted into the mouse kidney capsule and intra-adrenal. Importantly, the hypocortisolism of hiSCs derived from patients with adrenal insufficiency due to congenital adrenal hyperplasia was rescued by expressing the wild-type version of the defective disease-causing enzymes. Our study provides an effective tool with many potential applications for studying adrenal pathobiology in a personalized manner and opens venues for the development of precision therapies.

وصف الملف: application/pdf

العلاقة: info:eu-repo/semantics/altIdentifier/pmid/29386111; info:eu-repo/semantics/altIdentifier/wos/WOS:000423898500013; info:eu-repo/semantics/altIdentifier/isbn/2211-1247; info:eu-repo/semanti; https://push-zb.helmholtz-muenchen.de/frontdoor.php?source_opus=52861Test; urn:isbn:2211-1247; urn:issn:2211-1247

الإتاحة: https://doi.org/10.1016/j.celrep.2018.01.003Test
https://push-zb.helmholtz-muenchen.de/frontdoor.php?source_opus=52861Test

المؤلفون: Morin, Aurélie, Ruggiero, Carmen, Robidel, Estelle, Doghman-Bouguerra, Mabrouka, Das, Atze, T., Castellano, Rémy, Josselin, Emmanuelle, Favier, Judith, Lalli, Enzo

المساهمون: Virus, pseudo-virus: Morphogénèse et Antigénicité, Université de Tours (UT)-EA3856, Sorbonne Université (SU), Inserm UMR970, COMUE Université Côte d'Azur (2015-2019) (COMUE UCA), Centre de Recherche Cardiovasculaire de Lariboisiere, Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), AMC Liver Center, Academic Medical Center, Academic Medical Center - Academisch Medisch Centrum Amsterdam (AMC), University of Amsterdam Amsterdam = Universiteit van Amsterdam (UvA)-University of Amsterdam Amsterdam = Universiteit van Amsterdam (UvA), Laboratory of Experimental Virology - Department of Medical Microbiology Amsterdam, The Netherlands, University of Amsterdam Amsterdam = Universiteit van Amsterdam (UvA)-University of Amsterdam Amsterdam = Universiteit van Amsterdam (UvA)-Center for Infection and Immunity Amsterdam - CINIMA Amsterdam, The Netherlands, Centre de Recherche en Cancérologie de Marseille (CRCM), Aix Marseille Université (AMU)-Institut Paoli-Calmettes (IPC), Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université Paris Descartes - Paris 5 (UPD5), Institut de pharmacologie moléculaire et cellulaire (IPMC), Université Nice Sophia Antipolis (1965 - 2019) (UNS)-Centre National de la Recherche Scientifique (CNRS)-Université Côte d'Azur (UniCA)

المصدر: ISSN: 1949-2553 ; Oncotarget ; https://hal.science/hal-01788404Test ; Oncotarget, 2017.

مصطلحات موضوعية: GFP, epithelial-mesenchymal transition, EMT, adrenocortical carcinoma, HES, hematoxylin-eosin-safran, green fluorescent protein, adrenal cortex, cancer, cell lines, mouse models, xenografts Abbreviations: ACC, SF-1, Steroidogenic Factor 1, [SDV]Life Sciences [q-bio], [SDV.CAN]Life Sciences [q-bio]/Cancer

الوصف: International audience ; Adrenocortical carcinoma is a rare neoplasm with a poor prognosis. Very important advances have been made in the identification of the genetic determinants of adrenocortical carcinoma pathogenesis but our understanding is still limited about the mechanisms that determine cancer spread and metastasis. One major problem hindering preclinical experimentation for new therapies for adrenocortical carcinoma is represented by the lack of suitable animal models for metastatic disease. With the aim to overcome these limitations, in this study we tested several protocols in order to establish a mouse xenograft model of metastatic adrenocortical carcinoma. The most efficient method, based upon intrasplenic injection followed by splenectomy, produced metastases with high efficiency, whose development could be followed over time by bioluminescence measurements. We expect that the availability of this model will greatly improve the possibilities for preclinical testing of new treatments for advanced-stage disease.

العلاقة: hal-01788404; https://hal.science/hal-01788404Test; https://hal.science/hal-01788404/documentTest; https://hal.science/hal-01788404/file/establishment%20of%20a%20mouse.pdfTest

الإتاحة: https://hal.science/hal-01788404Test
https://hal.science/hal-01788404/documentTest
https://hal.science/hal-01788404/file/establishment%20of%20a%20mouse.pdfTest

المؤلفون: Alexander Blank, Robert W. Bendon, Halit Pinar, Laura Rabinowitz, Frido K. Bruehl, Amy Heerema-McKenney

المصدر: Pediatric and Developmental Pathology. 24:27-33

مصطلحات موضوعية: Pathology, medicine.medical_specialty, Placenta Diseases, Biopsy, Placenta, Choristoma, Steroidogenic Factor 1, Pathology and Forensic Medicine, Diagnosis, Differential, Antigens, Neoplasm, Predictive Value of Tests, Pregnancy, medicine, Humans, Arginase, business.industry, Cell Differentiation, General Medicine, Hepatic tissue, medicine.disease, Immunohistochemistry, Heterotopia (medicine), medicine.anatomical_structure, Liver, Pediatrics, Perinatology and Child Health, Adrenal Cortex, Female, Differential diagnosis, business

الوصف: Background Rare nodules of heterotopic adrenocortical and hepatic tissue are reported in the placenta. A mechanism for adrenocortical tissue in the placenta has been perplexing, while hepatic tissue is generally considered related to yolk sac primordia. The clear cell morphology of these nodules is similar to the adrenal cortex of the adult; however, the fetal adrenal gland does not usually display clear cells. Methods We stained 9 placental nodules, histologically identical to “adrenocortical” heterotopia of the placenta, to determine whether adrenocortical differentiation could be confirmed. These cases include 3 archival cases initially diagnosed as “adrenocortical” heterotopia. Results Immunohistochemical staining with steroid factor-1 (SF-1), HepPar-1, and Arginase-1 showed that these nodules of clear cells are actually hepatic (SF-1 negative, HepPar-1, and Arginase-1 positive). PAS staining suggests that glycogen accumulation is responsible for the clear cytoplasm. In contrast, a nodule of adrenocortical heterotopia near the testis and the adrenal gland from a 38-week-old neonatal autopsy case confirm SF-1 reactivity as expected. Conclusion We propose that adrenocortical heterotopia in the placenta is a misnomer, and that these subchorionic nodules of clear cells demonstrate hepatic differentiation.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::0153c6ad31ae81c43f74379248b9cbb3Test
https://doi.org/10.1177/1093526620953361Test

المؤلفون: Tarçın G., Ercan O.

مصطلحات موضوعية: adrenal rest, adrenocortical, choristoma, Ectopic adrenal, heterotopia, angiotensin, angiotensin II, corticotropin, cytochrome P450 family 21, glucocorticoid, luteinizing hormone, nuclear receptor DAX 1, steroidogenic factor 1, tetracosactide, transcription factor Hesx1, transcription factor LHX3, WT1 protein, adrenal cortex, adrenal gland, adrenal gland tissue, amenorrhea, androgen release, birth, brain hemorrhage, chromaffin cell, computer assisted tomography, conception, congenital adrenal hyperplasia, cyanosis, death

الوصف: Ectopic adrenal tissue, defined as the formation of adrenal tissue in an abnormal anatomical location, is not a rare entity and may have clinical significance. Even though the mechanism for their emergence has not been fully understood, numerous cases of ectopic adrenal tissue have been reported, mostly in the vicinity of the original location of adrenal gland, such as in kidneys and gonads. In these cases, most authors attributed their emergence to a probable migration defect. However, this mechanism does not simply explain the ectopic tissues in remote locations, such as in the hypophysis or lungs. This review summarizes these reports, describing many different locations in which ectopic adrenal tissues were encountered, together with their suggested mechanisms. © 2022 by Turkish Society for Pediatric Endocrinology and Diabetes.

العلاقة: JCRPE Journal of Clinical Research in Pediatric Endocrinology; Diğer; https://doi.org/10.4274/jcrpe.galenos.2021.2021.0148Test; https://hdl.handle.net/20.500.12831/9424Test; 14; 258; 266; 2-s2.0-85136255872

الإتاحة: https://doi.org/10.4274/jcrpe.galenos.2021.2021.0148Test
https://doi.org/20.500.12831/9424Test
https://hdl.handle.net/20.500.12831/9424Test

المؤلفون: Debora Bueno, Claudimara Ferini Pacicco Lotfi, Marco Aurélio Salomão Fortes, João A.B. Pedroso, Jose Donato, Angela M. Ramos-Lobo, Ismael Cabral Costa, Igor Tomaz, Isadora C. Furigo, Vitor L Pecorali, Thais Barabba Auricino, Pedro O. R. de Mendonca, Leandro B. Lima

المصدر: Repositório Institucional da USP (Biblioteca Digital da Produção Intelectual)
Universidade de São Paulo (USP)
instacron:USP

مصطلحات موضوعية: Male, 0301 basic medicine, Steroidogenic factor 1, endocrine system, medicine.medical_specialty, Endocrinology, Diabetes and Metabolism, medicine.medical_treatment, Deoxyglucose, Biology, Steroidogenic Factor 1, Mice, 03 medical and health sciences, chemistry.chemical_compound, Endocrinology, Corticosterone, Internal medicine, Adrenal Glands, Testis, medicine, Animals, Testosterone, SOCS3, Adiposity, Mice, Knockout, Adrenal gland, Adrenal cortex, digestive, oral, and skin physiology, Cell Differentiation, FISIOLOGIA, 030104 developmental biology, Ventromedial nucleus of the hypothalamus, medicine.anatomical_structure, Cytokine, chemistry, Suppressor of Cytokine Signaling 3 Protein, Pituitary Gland, Female, Hormone

الوصف: Many hormones/cytokines are secreted in response to exercise and cytokine signaling may play a pivotal role in the training adaptations. To investigate the importance of cytokine signaling during vertical ladder climbing, a resistance exercise model, we produced mice lacking SOCS3 protein exclusively in steroidogenic factor-1 (SF1) cells (SF1 Socs3 KO mice). SF1 expression is found in steroidogenic cells of the adrenal cortex and gonads, as well as in neurons of the ventromedial nucleus of the hypothalamus. Histological markers of the fetal adrenal zone (or X-zone in rodents) were still present in adult males and postpartum SF1 Socs3 KO females, suggesting a previously unrecognized effect of SOCS3 on the terminal differentiation of the adrenal gland. This change led to a distinct distribution of lipid droplets along the adrenal cortex. Under basal conditions, adult SF1 Socs3 KO mice exhibited similar adrenal weight, and plasma ACTH and corticosterone concentrations. Nonetheless, SF1 Socs3 KO mice exhibited a blunted ACTH-induced corticosterone secretion. The overall metabolic responses induced by resistance training remained unaffected in SF1 Socs3 KO mice, including changes in body adiposity, glucose tolerance and energy expenditure. However, training performance and glucose control during intense resistance exercise were impaired in SF1 Socs3 KO mice. Furthermore, a reduced counter-regulatory response to 2-deoxy-d-glucose was observed in mutant mice. These findings revealed a novel participation of SOCS3 regulating several endocrine and metabolic aspects. Therefore, cytokine signaling in SF1 cells exerts an important role to sustain training performance possibly by promoting the necessary metabolic adjustments during exercise.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8a3f3666c460542f201929220e9c0ec2Test
https://doi.org/10.1530/joe-17-0255Test

المؤلفون: Allan M. Judd, James P. Porter, S. McIlmoil, Janae Strickland, Brice J. Williams, Dennis C. Seager

المصدر: Steroids. 119:1-17

مصطلحات موضوعية: 0301 basic medicine, Steroidogenic factor 1, medicine.medical_specialty, Hydrocortisone, Blotting, Western, Clinical Biochemistry, 030209 endocrinology & metabolism, Biology, Steroidogenic Factor 1, Biochemistry, Cell Line, 03 medical and health sciences, 0302 clinical medicine, Endocrinology, Internal medicine, medicine, Humans, RNA, Messenger, STAT1, Phosphorylation, STAT3, Molecular Biology, Janus Kinases, Pharmacology, Interleukin-6, Cholesterol side-chain cleavage enzyme, Steroidogenic acute regulatory protein, Organic Chemistry, JAK-STAT signaling pathway, STAT Transcription Factors, 030104 developmental biology, Adrenal Cortex, STAT protein, biology.protein, Steroids, Transcription Factors

الوصف: Mechanisms of interleukin-6 (IL-6)-induced cortisol release (CR) were investigated by exposing H295R cells to IL-6 and determining mRNA/protein expression (PCR/western blots) for steroidogenic enzymes (SE), steroidogenic acute regulatory protein (StAR), steroidogenic factor-1 (SF-1) (enhances SE/StAR expression), activator protein 1 (AP-1) (regulates SE/StAR expression) and adrenal hypoplasia congenita-like protein (DAX-1) (inhibits SE/StAR expression). Promoter activity of StAR (SPA) was measured by a luciferase-coupled promoter. Cortisol release was increased by 10ng/mL IL-6 (24h P

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3cf9e10694d5414095c33e1fdbda0f6bTest
https://doi.org/10.1016/j.steroids.2016.12.014Test