Steroid profiling in male wobbler mouse, a model of Amyotrophic Lateral Sclerosis

Autores
Gonzalez Deniselle, Maria Claudia; Liere, Philippe; Pianos, Antoine; Meyer, Maria; Aprahamian, Fanny; Cambourg, Annie; Di Giorgio, Noelia Paula; Schumacher, Michael; de Nicola, Alejandro Federico; Guennoun, Rachida
Año de publicación
2016
Idioma
inglés
Tipo de recurso
artículo
Estado
versión publicada
Descripción
The Wobbler mouse is an animal model for human motoneuron diseases, especially amyotrophic lateral sclerosis (ALS), used in the investigation of both pathology and therapeutic treatment. ALS is a fatal neurodegenerative disease, characterized by the selective and progressive death of motoneurons, leading to progressive paralysis. Previous limited studies have reported steroidal hormone dysregulation in Wobbler mouse and in ALS patients, suggesting endocrine dysfunctions which may be involved in the pathogenesis of the disease. In this study, we established a steroid profiling in brain, spinal cord, plasma, adrenal glands, and testes in 2-month-old male Wobbler mice and their littermates by gas chromatography coupled to mass spectrometry. Our results show in Wobbler mice the following: 1) a marked up-regulation of corticosterone levels in adrenal glands, plasma, spinal cord regions (cervical, thoracic, lumbar) and brain; 2) a strong decrease in T levels in the testis, plasma, spinal cord, and brain; and 3) increased levels of progesterone and especially of its reduced metabolites 5α-dihydroprogesterone, allopregnanolone, and 20α-dihydroprogesterone in the brain, spinal cord, and adrenal glands. Furthermore, Wobbler mice showed a hypothalamic-pituitary-gonadal hypoactivity. Interestingly, plasma concentrations of corticosterone and T correlate well with their respective levels in cervical spinal cord in both control and Wobbler mice. T down-regulation is probably the consequence of adrenal hyperactivity, and the up-regulation of progesterone and its reduced metabolites may correspond to an endogenous protective mechanism in response to motoneuron degeneration. Our findings suggest that increased levels of corticosterone and decreased levels of T in plasma could be a signature of motoneuron degeneration.
Fil: Gonzalez Deniselle, Maria Claudia. Universidad de Buenos Aires. Facultad de Medicina; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Biología y Medicina Experimental. Fundación de Instituto de Biología y Medicina Experimental. Instituto de Biología y Medicina Experimental; Argentina
Fil: Liere, Philippe. Inserm; Francia. Université Paris Saclay; Francia
Fil: Pianos, Antoine. Inserm; Francia. Université Paris Saclay; Francia
Fil: Meyer, Maria. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Biología y Medicina Experimental. Fundación de Instituto de Biología y Medicina Experimental. Instituto de Biología y Medicina Experimental; Argentina
Fil: Aprahamian, Fanny. Inserm; Francia. Université Paris Saclay; Francia
Fil: Cambourg, Annie. Inserm; Francia. Université Paris Saclay; Francia
Fil: Di Giorgio, Noelia Paula. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Biología y Medicina Experimental. Fundación de Instituto de Biología y Medicina Experimental. Instituto de Biología y Medicina Experimental; Argentina
Fil: Schumacher, Michael. Inserm; Francia. Université Paris Saclay; Francia. Universite Paris Sud; Francia
Fil: de Nicola, Alejandro Federico. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Biología y Medicina Experimental. Fundación de Instituto de Biología y Medicina Experimental. Instituto de Biología y Medicina Experimental; Argentina
Fil: Guennoun, Rachida. Université Paris Saclay; Francia. Inserm; Francia. Universite Paris Sud; Francia
Materia
STEROIDS
MICE
AMYTROPHIC LATERAL SCLEROSIS
BRAIN
CORTICOSTERONE
PROGESTERONE
Nivel de accesibilidad
acceso embargado
Condiciones de uso
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
Repositorio
CONICET Digital (CONICET)
Institución
Consejo Nacional de Investigaciones Científicas y Técnicas
OAI Identificador
oai:ri.conicet.gov.ar:11336/24577
Acceder
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oai_identifier_str oai:ri.conicet.gov.ar:11336/24577
network_acronym_str CONICETDig
repository_id_str 3498
network_name_str CONICET Digital (CONICET)
spelling Steroid profiling in male wobbler mouse, a model of Amyotrophic Lateral SclerosisGonzalez Deniselle, Maria ClaudiaLiere, PhilippePianos, AntoineMeyer, MariaAprahamian, FannyCambourg, AnnieDi Giorgio, Noelia PaulaSchumacher, Michaelde Nicola, Alejandro FedericoGuennoun, RachidaSTEROIDSMICEAMYTROPHIC LATERAL SCLEROSISBRAINCORTICOSTERONEPROGESTERONEhttps://purl.org/becyt/ford/3.1https://purl.org/becyt/ford/3https://purl.org/becyt/ford/3.1https://purl.org/becyt/ford/3The Wobbler mouse is an animal model for human motoneuron diseases, especially amyotrophic lateral sclerosis (ALS), used in the investigation of both pathology and therapeutic treatment. ALS is a fatal neurodegenerative disease, characterized by the selective and progressive death of motoneurons, leading to progressive paralysis. Previous limited studies have reported steroidal hormone dysregulation in Wobbler mouse and in ALS patients, suggesting endocrine dysfunctions which may be involved in the pathogenesis of the disease. In this study, we established a steroid profiling in brain, spinal cord, plasma, adrenal glands, and testes in 2-month-old male Wobbler mice and their littermates by gas chromatography coupled to mass spectrometry. Our results show in Wobbler mice the following: 1) a marked up-regulation of corticosterone levels in adrenal glands, plasma, spinal cord regions (cervical, thoracic, lumbar) and brain; 2) a strong decrease in T levels in the testis, plasma, spinal cord, and brain; and 3) increased levels of progesterone and especially of its reduced metabolites 5α-dihydroprogesterone, allopregnanolone, and 20α-dihydroprogesterone in the brain, spinal cord, and adrenal glands. Furthermore, Wobbler mice showed a hypothalamic-pituitary-gonadal hypoactivity. Interestingly, plasma concentrations of corticosterone and T correlate well with their respective levels in cervical spinal cord in both control and Wobbler mice. T down-regulation is probably the consequence of adrenal hyperactivity, and the up-regulation of progesterone and its reduced metabolites may correspond to an endogenous protective mechanism in response to motoneuron degeneration. Our findings suggest that increased levels of corticosterone and decreased levels of T in plasma could be a signature of motoneuron degeneration.Fil: Gonzalez Deniselle, Maria Claudia. Universidad de Buenos Aires. Facultad de Medicina; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Biología y Medicina Experimental. Fundación de Instituto de Biología y Medicina Experimental. Instituto de Biología y Medicina Experimental; ArgentinaFil: Liere, Philippe. Inserm; Francia. Université Paris Saclay; FranciaFil: Pianos, Antoine. Inserm; Francia. Université Paris Saclay; FranciaFil: Meyer, Maria. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Biología y Medicina Experimental. Fundación de Instituto de Biología y Medicina Experimental. Instituto de Biología y Medicina Experimental; ArgentinaFil: Aprahamian, Fanny. Inserm; Francia. Université Paris Saclay; FranciaFil: Cambourg, Annie. Inserm; Francia. Université Paris Saclay; FranciaFil: Di Giorgio, Noelia Paula. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Biología y Medicina Experimental. Fundación de Instituto de Biología y Medicina Experimental. Instituto de Biología y Medicina Experimental; ArgentinaFil: Schumacher, Michael. Inserm; Francia. Université Paris Saclay; Francia. Universite Paris Sud; FranciaFil: de Nicola, Alejandro Federico. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Biología y Medicina Experimental. Fundación de Instituto de Biología y Medicina Experimental. Instituto de Biología y Medicina Experimental; ArgentinaFil: Guennoun, Rachida. Université Paris Saclay; Francia. Inserm; Francia. Universite Paris Sud; FranciaEndocrine Society2016-11-01info:eu-repo/date/embargoEnd/2017-11-02info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/pdfapplication/pdfapplication/pdfhttp://hdl.handle.net/11336/24577Gonzalez Deniselle, Maria Claudia; Liere, Philippe; Pianos, Antoine; Meyer, Maria; Aprahamian, Fanny; et al.; Steroid profiling in male wobbler mouse, a model of Amyotrophic Lateral Sclerosis; Endocrine Society; Endocrinology; 157; 11; 1-11-2016; 4446-44600013-72271945-7170CONICET DigitalCONICETenginfo:eu-repo/semantics/altIdentifier/url/https://academic.oup.com/endo/article-abstract/157/11/4446/2758423/Steroid-Profiling-in-Male-Wobbler-Mouse-a-Model-of?redirectedFrom=fulltextinfo:eu-repo/semantics/altIdentifier/doi/10.1210/en.2016-1244info:eu-repo/semantics/altIdentifier/pmid/27571131info:eu-repo/semantics/embargoedAccesshttps://creativecommons.org/licenses/by-nc-sa/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-09-03T09:51:14Zoai:ri.conicet.gov.ar:11336/24577instacron:CONICETInstitucionalhttp://ri.conicet.gov.ar/Organismo científico-tecnológicoNo correspondehttp://ri.conicet.gov.ar/oai/requestdasensio@conicet.gov.ar; lcarlino@conicet.gov.arArgentinaNo correspondeNo correspondeNo correspondeopendoar:34982025-09-03 09:51:15.052CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse
dc.title.none.fl_str_mv Steroid profiling in male wobbler mouse, a model of Amyotrophic Lateral Sclerosis
title Steroid profiling in male wobbler mouse, a model of Amyotrophic Lateral Sclerosis
spellingShingle Steroid profiling in male wobbler mouse, a model of Amyotrophic Lateral Sclerosis
Gonzalez Deniselle, Maria Claudia
STEROIDS
MICE
AMYTROPHIC LATERAL SCLEROSIS
BRAIN
CORTICOSTERONE
PROGESTERONE
title_short Steroid profiling in male wobbler mouse, a model of Amyotrophic Lateral Sclerosis
title_full Steroid profiling in male wobbler mouse, a model of Amyotrophic Lateral Sclerosis
title_fullStr Steroid profiling in male wobbler mouse, a model of Amyotrophic Lateral Sclerosis
title_full_unstemmed Steroid profiling in male wobbler mouse, a model of Amyotrophic Lateral Sclerosis
title_sort Steroid profiling in male wobbler mouse, a model of Amyotrophic Lateral Sclerosis
dc.creator.none.fl_str_mv Gonzalez Deniselle, Maria Claudia
Liere, Philippe
Pianos, Antoine
Meyer, Maria
Aprahamian, Fanny
Cambourg, Annie
Di Giorgio, Noelia Paula
Schumacher, Michael
de Nicola, Alejandro Federico
Guennoun, Rachida
author Gonzalez Deniselle, Maria Claudia
author_facet Gonzalez Deniselle, Maria Claudia
Liere, Philippe
Pianos, Antoine
Meyer, Maria
Aprahamian, Fanny
Cambourg, Annie
Di Giorgio, Noelia Paula
Schumacher, Michael
de Nicola, Alejandro Federico
Guennoun, Rachida
author_role author
author2 Liere, Philippe
Pianos, Antoine
Meyer, Maria
Aprahamian, Fanny
Cambourg, Annie
Di Giorgio, Noelia Paula
Schumacher, Michael
de Nicola, Alejandro Federico
Guennoun, Rachida
author2_role author
author
author
author
author
author
author
author
author
dc.subject.none.fl_str_mv STEROIDS
MICE
AMYTROPHIC LATERAL SCLEROSIS
BRAIN
CORTICOSTERONE
PROGESTERONE
topic STEROIDS
MICE
AMYTROPHIC LATERAL SCLEROSIS
BRAIN
CORTICOSTERONE
PROGESTERONE
purl_subject.fl_str_mv https://purl.org/becyt/ford/3.1
https://purl.org/becyt/ford/3
https://purl.org/becyt/ford/3.1
https://purl.org/becyt/ford/3
dc.description.none.fl_txt_mv The Wobbler mouse is an animal model for human motoneuron diseases, especially amyotrophic lateral sclerosis (ALS), used in the investigation of both pathology and therapeutic treatment. ALS is a fatal neurodegenerative disease, characterized by the selective and progressive death of motoneurons, leading to progressive paralysis. Previous limited studies have reported steroidal hormone dysregulation in Wobbler mouse and in ALS patients, suggesting endocrine dysfunctions which may be involved in the pathogenesis of the disease. In this study, we established a steroid profiling in brain, spinal cord, plasma, adrenal glands, and testes in 2-month-old male Wobbler mice and their littermates by gas chromatography coupled to mass spectrometry. Our results show in Wobbler mice the following: 1) a marked up-regulation of corticosterone levels in adrenal glands, plasma, spinal cord regions (cervical, thoracic, lumbar) and brain; 2) a strong decrease in T levels in the testis, plasma, spinal cord, and brain; and 3) increased levels of progesterone and especially of its reduced metabolites 5α-dihydroprogesterone, allopregnanolone, and 20α-dihydroprogesterone in the brain, spinal cord, and adrenal glands. Furthermore, Wobbler mice showed a hypothalamic-pituitary-gonadal hypoactivity. Interestingly, plasma concentrations of corticosterone and T correlate well with their respective levels in cervical spinal cord in both control and Wobbler mice. T down-regulation is probably the consequence of adrenal hyperactivity, and the up-regulation of progesterone and its reduced metabolites may correspond to an endogenous protective mechanism in response to motoneuron degeneration. Our findings suggest that increased levels of corticosterone and decreased levels of T in plasma could be a signature of motoneuron degeneration.
Fil: Gonzalez Deniselle, Maria Claudia. Universidad de Buenos Aires. Facultad de Medicina; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Biología y Medicina Experimental. Fundación de Instituto de Biología y Medicina Experimental. Instituto de Biología y Medicina Experimental; Argentina
Fil: Liere, Philippe. Inserm; Francia. Université Paris Saclay; Francia
Fil: Pianos, Antoine. Inserm; Francia. Université Paris Saclay; Francia
Fil: Meyer, Maria. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Biología y Medicina Experimental. Fundación de Instituto de Biología y Medicina Experimental. Instituto de Biología y Medicina Experimental; Argentina
Fil: Aprahamian, Fanny. Inserm; Francia. Université Paris Saclay; Francia
Fil: Cambourg, Annie. Inserm; Francia. Université Paris Saclay; Francia
Fil: Di Giorgio, Noelia Paula. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Biología y Medicina Experimental. Fundación de Instituto de Biología y Medicina Experimental. Instituto de Biología y Medicina Experimental; Argentina
Fil: Schumacher, Michael. Inserm; Francia. Université Paris Saclay; Francia. Universite Paris Sud; Francia
Fil: de Nicola, Alejandro Federico. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Biología y Medicina Experimental. Fundación de Instituto de Biología y Medicina Experimental. Instituto de Biología y Medicina Experimental; Argentina
Fil: Guennoun, Rachida. Université Paris Saclay; Francia. Inserm; Francia. Universite Paris Sud; Francia
description The Wobbler mouse is an animal model for human motoneuron diseases, especially amyotrophic lateral sclerosis (ALS), used in the investigation of both pathology and therapeutic treatment. ALS is a fatal neurodegenerative disease, characterized by the selective and progressive death of motoneurons, leading to progressive paralysis. Previous limited studies have reported steroidal hormone dysregulation in Wobbler mouse and in ALS patients, suggesting endocrine dysfunctions which may be involved in the pathogenesis of the disease. In this study, we established a steroid profiling in brain, spinal cord, plasma, adrenal glands, and testes in 2-month-old male Wobbler mice and their littermates by gas chromatography coupled to mass spectrometry. Our results show in Wobbler mice the following: 1) a marked up-regulation of corticosterone levels in adrenal glands, plasma, spinal cord regions (cervical, thoracic, lumbar) and brain; 2) a strong decrease in T levels in the testis, plasma, spinal cord, and brain; and 3) increased levels of progesterone and especially of its reduced metabolites 5α-dihydroprogesterone, allopregnanolone, and 20α-dihydroprogesterone in the brain, spinal cord, and adrenal glands. Furthermore, Wobbler mice showed a hypothalamic-pituitary-gonadal hypoactivity. Interestingly, plasma concentrations of corticosterone and T correlate well with their respective levels in cervical spinal cord in both control and Wobbler mice. T down-regulation is probably the consequence of adrenal hyperactivity, and the up-regulation of progesterone and its reduced metabolites may correspond to an endogenous protective mechanism in response to motoneuron degeneration. Our findings suggest that increased levels of corticosterone and decreased levels of T in plasma could be a signature of motoneuron degeneration.
publishDate 2016
dc.date.none.fl_str_mv 2016-11-01
info:eu-repo/date/embargoEnd/2017-11-02
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
http://purl.org/coar/resource_type/c_6501
info:ar-repo/semantics/articulo
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/11336/24577
Gonzalez Deniselle, Maria Claudia; Liere, Philippe; Pianos, Antoine; Meyer, Maria; Aprahamian, Fanny; et al.; Steroid profiling in male wobbler mouse, a model of Amyotrophic Lateral Sclerosis; Endocrine Society; Endocrinology; 157; 11; 1-11-2016; 4446-4460
0013-7227
1945-7170
CONICET Digital
CONICET
url http://hdl.handle.net/11336/24577
identifier_str_mv Gonzalez Deniselle, Maria Claudia; Liere, Philippe; Pianos, Antoine; Meyer, Maria; Aprahamian, Fanny; et al.; Steroid profiling in male wobbler mouse, a model of Amyotrophic Lateral Sclerosis; Endocrine Society; Endocrinology; 157; 11; 1-11-2016; 4446-4460
0013-7227
1945-7170
CONICET Digital
CONICET
dc.language.none.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv info:eu-repo/semantics/altIdentifier/url/https://academic.oup.com/endo/article-abstract/157/11/4446/2758423/Steroid-Profiling-in-Male-Wobbler-Mouse-a-Model-of?redirectedFrom=fulltext
info:eu-repo/semantics/altIdentifier/doi/10.1210/en.2016-1244
info:eu-repo/semantics/altIdentifier/pmid/27571131
dc.rights.none.fl_str_mv info:eu-repo/semantics/embargoedAccess
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
eu_rights_str_mv embargoedAccess
rights_invalid_str_mv https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
dc.format.none.fl_str_mv application/pdf
application/pdf
application/pdf
application/pdf
dc.publisher.none.fl_str_mv Endocrine Society
publisher.none.fl_str_mv Endocrine Society
dc.source.none.fl_str_mv reponame:CONICET Digital (CONICET)
instname:Consejo Nacional de Investigaciones Científicas y Técnicas
reponame_str CONICET Digital (CONICET)
collection CONICET Digital (CONICET)
instname_str Consejo Nacional de Investigaciones Científicas y Técnicas
repository.name.fl_str_mv CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicas
repository.mail.fl_str_mv dasensio@conicet.gov.ar; lcarlino@conicet.gov.ar
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score 13.13397

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