Uncoupling of osteoblast-osteoclast regulation in a chemical murine model of Gaucher disease

Autores
Mucci, Juan Marcos; Suqueli García, María Florencia; de Francesco, Pablo Nicolás; Ceci, Romina; Di Genaro, S.; Fossati, Carlos Alberto; Delpino, María Victoria; Rozenfeld, Paula Adriana
Año de publicación
2013
Idioma
inglés
Tipo de recurso
artículo
Estado
versión publicada
Descripción
Gaucher disease (GD) is caused by mutations in the GBA gene that confer a deficient level of activity of glucocerebrosidase (GCase). This deficiency leads to accumulation of the glycolipid glucocerebroside in the lysosomes of cells ofmonocyte/macrophage system. Type I GDis the mildest formand is characterized by the absence of neuronopathic affection. Bone compromise in Gaucher disease patients is the most disabling aspect of the disease. However, pathophysiological aspects of skeletal alterations are still poorly understood. The homeostasis of bone tissue ismaintained by the balanced processes of bone resorption by osteoclasts and formation
by osteoblasts. We decided to test whether bone resorption and/or bone formation could be altered by the use of a chemical in vitro murine model of Gaucher disease.
We used two sources of cells from monocyte/macrophages lineage isolated from normal mice, splenocytes (S) and peritonealmacrophages (PM), and were exposed to CBE, the inhibitor of GCase (S-CBE and PM-CBE, respectively).
Addition of both conditioned media (CM) from S-CBE and PM-CBE induced the differentiation of osteoclasts precursors from bone marrow to mature and functional osteoclasts. TNF-α could be one of the factors responsible for this effect. On the other hand, addition of CM to an osteoblast cell culture resulted in a reduction in expression of alkaline phosphatase and mineralization process. In conclusion, these results suggest implication of changes in both bone formation and bone resorption and are consistent with the idea that both sides of the homeostatic balance are affected in GD
Fil: Mucci, Juan Marcos. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Universidad Nacional de la Plata. Facultad de Ciencias Exactas. Departamento de Ciencias Biologicas. Laboratorio de Investigaciones del Sistema Inmune; Argentina
Fil: Suqueli García, María Florencia. Universidad Nacional de la Plata. Facultad de Ciencias Exactas. Departamento de Ciencias Biologicas. Laboratorio de Investigaciones del Sistema Inmune; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
Fil: de Francesco, Pablo Nicolás. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Universidad Nacional de la Plata. Facultad de Ciencias Exactas. Departamento de Ciencias Biologicas. Laboratorio de Investigaciones del Sistema Inmune; Argentina
Fil: Ceci, Romina. Universidad Nacional de la Plata. Facultad de Ciencias Exactas. Departamento de Ciencias Biologicas. Laboratorio de Investigaciones del Sistema Inmune; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
Fil: Di Genaro, S.. Universidad Nacional de San Luis; Argentina
Fil: Fossati, Carlos Alberto. Universidad Nacional de la Plata. Facultad de Ciencias Exactas. Departamento de Ciencias Biologicas. Laboratorio de Investigaciones del Sistema Inmune; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
Fil: Delpino, María Victoria. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Inmunología, Genética y Metabolismo; Argentina. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina
Fil: Rozenfeld, Paula Adriana. Universidad Nacional de la Plata. Facultad de Ciencias Exactas. Departamento de Ciencias Biologicas. Laboratorio de Investigaciones del Sistema Inmune; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
Materia
Gaucher
Osteoblast
Osteoclast
Rankl
Nivel de accesibilidad
acceso abierto
Condiciones de uso
https://creativecommons.org/licenses/by-nc-nd/2.5/ar/
Repositorio
CONICET Digital (CONICET)
Institución
Consejo Nacional de Investigaciones Científicas y Técnicas
OAI Identificador
oai:ri.conicet.gov.ar:11336/12441
Acceder
id CONICETDig_f5a02a91d02fb7f90ded217ae1469d97
oai_identifier_str oai:ri.conicet.gov.ar:11336/12441
network_acronym_str CONICETDig
repository_id_str 3498
network_name_str CONICET Digital (CONICET)
spelling Uncoupling of osteoblast-osteoclast regulation in a chemical murine model of Gaucher diseaseMucci, Juan MarcosSuqueli García, María Florenciade Francesco, Pablo NicolásCeci, RominaDi Genaro, S.Fossati, Carlos AlbertoDelpino, María VictoriaRozenfeld, Paula AdrianaGaucherOsteoblastOsteoclastRanklhttps://purl.org/becyt/ford/3.1https://purl.org/becyt/ford/3Gaucher disease (GD) is caused by mutations in the GBA gene that confer a deficient level of activity of glucocerebrosidase (GCase). This deficiency leads to accumulation of the glycolipid glucocerebroside in the lysosomes of cells ofmonocyte/macrophage system. Type I GDis the mildest formand is characterized by the absence of neuronopathic affection. Bone compromise in Gaucher disease patients is the most disabling aspect of the disease. However, pathophysiological aspects of skeletal alterations are still poorly understood. The homeostasis of bone tissue ismaintained by the balanced processes of bone resorption by osteoclasts and formation<br />by osteoblasts. We decided to test whether bone resorption and/or bone formation could be altered by the use of a chemical in vitro murine model of Gaucher disease.<br />We used two sources of cells from monocyte/macrophages lineage isolated from normal mice, splenocytes (S) and peritonealmacrophages (PM), and were exposed to CBE, the inhibitor of GCase (S-CBE and PM-CBE, respectively).<br />Addition of both conditioned media (CM) from S-CBE and PM-CBE induced the differentiation of osteoclasts precursors from bone marrow to mature and functional osteoclasts. TNF-α could be one of the factors responsible for this effect. On the other hand, addition of CM to an osteoblast cell culture resulted in a reduction in expression of alkaline phosphatase and mineralization process. In conclusion, these results suggest implication of changes in both bone formation and bone resorption and are consistent with the idea that both sides of the homeostatic balance are affected in GDFil: Mucci, Juan Marcos. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Universidad Nacional de la Plata. Facultad de Ciencias Exactas. Departamento de Ciencias Biologicas. Laboratorio de Investigaciones del Sistema Inmune; ArgentinaFil: Suqueli García, María Florencia. Universidad Nacional de la Plata. Facultad de Ciencias Exactas. Departamento de Ciencias Biologicas. Laboratorio de Investigaciones del Sistema Inmune; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: de Francesco, Pablo Nicolás. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Universidad Nacional de la Plata. Facultad de Ciencias Exactas. Departamento de Ciencias Biologicas. Laboratorio de Investigaciones del Sistema Inmune; ArgentinaFil: Ceci, Romina. Universidad Nacional de la Plata. Facultad de Ciencias Exactas. Departamento de Ciencias Biologicas. Laboratorio de Investigaciones del Sistema Inmune; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: Di Genaro, S.. Universidad Nacional de San Luis; ArgentinaFil: Fossati, Carlos Alberto. Universidad Nacional de la Plata. Facultad de Ciencias Exactas. Departamento de Ciencias Biologicas. Laboratorio de Investigaciones del Sistema Inmune; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: Delpino, María Victoria. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Inmunología, Genética y Metabolismo; Argentina. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; ArgentinaFil: Rozenfeld, Paula Adriana. Universidad Nacional de la Plata. Facultad de Ciencias Exactas. Departamento de Ciencias Biologicas. Laboratorio de Investigaciones del Sistema Inmune; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaElsevier Science2013-10info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/pdfapplication/pdfhttp://hdl.handle.net/11336/12441Mucci, Juan Marcos; Suqueli García, María Florencia; de Francesco, Pablo Nicolás; Ceci, Romina; Di Genaro, S.; et al.; Uncoupling of osteoblast-osteoclast regulation in a chemical murine model of Gaucher disease; Elsevier Science; Gene; 532; 2; 10-2013; 186-1910378-1119enginfo:eu-repo/semantics/altIdentifier/url/http://www.sciencedirect.com/science/article/pii/S0378111913012924info:eu-repo/semantics/altIdentifier/doi/10.1016/j.gene.2013.09.072info:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by-nc-nd/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-10-15T14:45:16Zoai:ri.conicet.gov.ar:11336/12441instacron: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:34982025-10-15 14:45:16.926CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse
dc.title.none.fl_str_mv Uncoupling of osteoblast-osteoclast regulation in a chemical murine model of Gaucher disease
title Uncoupling of osteoblast-osteoclast regulation in a chemical murine model of Gaucher disease
spellingShingle Uncoupling of osteoblast-osteoclast regulation in a chemical murine model of Gaucher disease
Mucci, Juan Marcos
Gaucher
Osteoblast
Osteoclast
Rankl
title_short Uncoupling of osteoblast-osteoclast regulation in a chemical murine model of Gaucher disease
title_full Uncoupling of osteoblast-osteoclast regulation in a chemical murine model of Gaucher disease
title_fullStr Uncoupling of osteoblast-osteoclast regulation in a chemical murine model of Gaucher disease
title_full_unstemmed Uncoupling of osteoblast-osteoclast regulation in a chemical murine model of Gaucher disease
title_sort Uncoupling of osteoblast-osteoclast regulation in a chemical murine model of Gaucher disease
dc.creator.none.fl_str_mv Mucci, Juan Marcos
Suqueli García, María Florencia
de Francesco, Pablo Nicolás
Ceci, Romina
Di Genaro, S.
Fossati, Carlos Alberto
Delpino, María Victoria
Rozenfeld, Paula Adriana
author Mucci, Juan Marcos
author_facet Mucci, Juan Marcos
Suqueli García, María Florencia
de Francesco, Pablo Nicolás
Ceci, Romina
Di Genaro, S.
Fossati, Carlos Alberto
Delpino, María Victoria
Rozenfeld, Paula Adriana
author_role author
author2 Suqueli García, María Florencia
de Francesco, Pablo Nicolás
Ceci, Romina
Di Genaro, S.
Fossati, Carlos Alberto
Delpino, María Victoria
Rozenfeld, Paula Adriana
author2_role author
author
author
author
author
author
author
dc.subject.none.fl_str_mv Gaucher
Osteoblast
Osteoclast
Rankl
topic Gaucher
Osteoblast
Osteoclast
Rankl
purl_subject.fl_str_mv https://purl.org/becyt/ford/3.1
https://purl.org/becyt/ford/3
dc.description.none.fl_txt_mv Gaucher disease (GD) is caused by mutations in the GBA gene that confer a deficient level of activity of glucocerebrosidase (GCase). This deficiency leads to accumulation of the glycolipid glucocerebroside in the lysosomes of cells ofmonocyte/macrophage system. Type I GDis the mildest formand is characterized by the absence of neuronopathic affection. Bone compromise in Gaucher disease patients is the most disabling aspect of the disease. However, pathophysiological aspects of skeletal alterations are still poorly understood. The homeostasis of bone tissue ismaintained by the balanced processes of bone resorption by osteoclasts and formation<br />by osteoblasts. We decided to test whether bone resorption and/or bone formation could be altered by the use of a chemical in vitro murine model of Gaucher disease.<br />We used two sources of cells from monocyte/macrophages lineage isolated from normal mice, splenocytes (S) and peritonealmacrophages (PM), and were exposed to CBE, the inhibitor of GCase (S-CBE and PM-CBE, respectively).<br />Addition of both conditioned media (CM) from S-CBE and PM-CBE induced the differentiation of osteoclasts precursors from bone marrow to mature and functional osteoclasts. TNF-α could be one of the factors responsible for this effect. On the other hand, addition of CM to an osteoblast cell culture resulted in a reduction in expression of alkaline phosphatase and mineralization process. In conclusion, these results suggest implication of changes in both bone formation and bone resorption and are consistent with the idea that both sides of the homeostatic balance are affected in GD
Fil: Mucci, Juan Marcos. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Universidad Nacional de la Plata. Facultad de Ciencias Exactas. Departamento de Ciencias Biologicas. Laboratorio de Investigaciones del Sistema Inmune; Argentina
Fil: Suqueli García, María Florencia. Universidad Nacional de la Plata. Facultad de Ciencias Exactas. Departamento de Ciencias Biologicas. Laboratorio de Investigaciones del Sistema Inmune; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
Fil: de Francesco, Pablo Nicolás. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Universidad Nacional de la Plata. Facultad de Ciencias Exactas. Departamento de Ciencias Biologicas. Laboratorio de Investigaciones del Sistema Inmune; Argentina
Fil: Ceci, Romina. Universidad Nacional de la Plata. Facultad de Ciencias Exactas. Departamento de Ciencias Biologicas. Laboratorio de Investigaciones del Sistema Inmune; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
Fil: Di Genaro, S.. Universidad Nacional de San Luis; Argentina
Fil: Fossati, Carlos Alberto. Universidad Nacional de la Plata. Facultad de Ciencias Exactas. Departamento de Ciencias Biologicas. Laboratorio de Investigaciones del Sistema Inmune; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
Fil: Delpino, María Victoria. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Inmunología, Genética y Metabolismo; Argentina. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina
Fil: Rozenfeld, Paula Adriana. Universidad Nacional de la Plata. Facultad de Ciencias Exactas. Departamento de Ciencias Biologicas. Laboratorio de Investigaciones del Sistema Inmune; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
description Gaucher disease (GD) is caused by mutations in the GBA gene that confer a deficient level of activity of glucocerebrosidase (GCase). This deficiency leads to accumulation of the glycolipid glucocerebroside in the lysosomes of cells ofmonocyte/macrophage system. Type I GDis the mildest formand is characterized by the absence of neuronopathic affection. Bone compromise in Gaucher disease patients is the most disabling aspect of the disease. However, pathophysiological aspects of skeletal alterations are still poorly understood. The homeostasis of bone tissue ismaintained by the balanced processes of bone resorption by osteoclasts and formation<br />by osteoblasts. We decided to test whether bone resorption and/or bone formation could be altered by the use of a chemical in vitro murine model of Gaucher disease.<br />We used two sources of cells from monocyte/macrophages lineage isolated from normal mice, splenocytes (S) and peritonealmacrophages (PM), and were exposed to CBE, the inhibitor of GCase (S-CBE and PM-CBE, respectively).<br />Addition of both conditioned media (CM) from S-CBE and PM-CBE induced the differentiation of osteoclasts precursors from bone marrow to mature and functional osteoclasts. TNF-α could be one of the factors responsible for this effect. On the other hand, addition of CM to an osteoblast cell culture resulted in a reduction in expression of alkaline phosphatase and mineralization process. In conclusion, these results suggest implication of changes in both bone formation and bone resorption and are consistent with the idea that both sides of the homeostatic balance are affected in GD
publishDate 2013
dc.date.none.fl_str_mv 2013-10
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/11336/12441
Mucci, Juan Marcos; Suqueli García, María Florencia; de Francesco, Pablo Nicolás; Ceci, Romina; Di Genaro, S.; et al.; Uncoupling of osteoblast-osteoclast regulation in a chemical murine model of Gaucher disease; Elsevier Science; Gene; 532; 2; 10-2013; 186-191
0378-1119
url http://hdl.handle.net/11336/12441
identifier_str_mv Mucci, Juan Marcos; Suqueli García, María Florencia; de Francesco, Pablo Nicolás; Ceci, Romina; Di Genaro, S.; et al.; Uncoupling of osteoblast-osteoclast regulation in a chemical murine model of Gaucher disease; Elsevier Science; Gene; 532; 2; 10-2013; 186-191
0378-1119
dc.language.none.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv info:eu-repo/semantics/altIdentifier/url/http://www.sciencedirect.com/science/article/pii/S0378111913012924
info:eu-repo/semantics/altIdentifier/doi/10.1016/j.gene.2013.09.072
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
https://creativecommons.org/licenses/by-nc-nd/2.5/ar/
eu_rights_str_mv openAccess
rights_invalid_str_mv https://creativecommons.org/licenses/by-nc-nd/2.5/ar/
dc.format.none.fl_str_mv application/pdf
application/pdf
application/pdf
dc.publisher.none.fl_str_mv Elsevier Science
publisher.none.fl_str_mv Elsevier Science
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 13.22299

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