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dc.date.accessioned 2023-11-16T18:12:33Z
dc.date.available 2023-11-16T18:12:33Z
dc.date.issued 2023
dc.identifier.uri http://sedici.unlp.edu.ar/handle/10915/160261
dc.description.abstract Gaucher disease (GD) is caused by biallelic pathogenic variants in GBA1 gene that encodes the lysosomal enzyme glucocerebrosidase. Up to now, specific treatment for GD cannot completely reverse bone complications. Bone is composed of different cell types; including osteoblasts, osteocytes and osteoclasts. Osteoblasts are present on bone surfaces and are derived from local mesenchymal stem cells (MSCs). Depending on environment conditions, MSCs could differentiate into osteoblasts and adipocytes. Mature adipocytes-secreted adipokines and free fatty acids affect both osteoblasts and osteoclasts formation/activity and therefore mediate skeletal homeostasis. The aim of this study was to evaluate possible alterations in GD adipocyte (GD Ad) that could contribute to bone complications. MSCs were grown in adipogenic media in order to evaluate expression of differentiation markers as PPAR-γ. PPAR-γ was observed into the nucleus of GD Ad, indicating that these cells are properly stimulated. However, these cells accumulate lesser lipid droplets (LDs) than Control Ad. In order to study lipid droplet metabolism, we evaluated the lipolysis of these structures by the measurement of free glycerol in culture supernatant. Our results indicated that GD Ad had an alteration in this process, evidenced by an increase in glycerol release. We have also evaluated two enzymes involved in LDs synthesis: fatty acid synthase (FASN) and stearoylcoenzyme A desaturase 1 (SCD1). The transcription of these genes was decreased in GD Ad, suggesting a dysfunction in the synthesis of LDs. In conclusion, our results show an alteration in LDs metabolism of GD Ad, independent of adipocyte differentiation process. This alteration would be caused by an increase in lipolysis in early stages of differentiation and also by a reduction of lipid synthesis, which could contribute with the skeletal imbalance in GD. en
dc.language en es
dc.subject Gaucher es
dc.subject MSCs es
dc.subject Adipocytes es
dc.subject Bone es
dc.subject Lipid droplets es
dc.title Bone marrow adipocytes alteration in an in vitro model of Gaucher Disease en
dc.type Articulo es
sedici.identifier.other https://doi.org/10.1016/j.ymgmr.2023.100980 es
sedici.identifier.issn 2214-4269 es
sedici.creator.person Crivaro, Andrea Natalia es
sedici.creator.person Mucci, Juan Marcos es
sedici.creator.person Bondar, Constanza María es
sedici.creator.person Ormazabal, Maximiliano Emanuel es
sedici.creator.person Vaena, Emilio es
sedici.creator.person Delpino, María Victoria es
sedici.subject.materias Biología es
sedici.description.fulltext true es
mods.originInfo.place Instituto de Estudios Inmunológicos y Fisiopatológicos es
sedici.subtype Articulo es
sedici.rights.license Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0)
sedici.rights.uri http://creativecommons.org/licenses/by-nc-nd/4.0/
sedici.description.peerReview peer-review es
sedici.relation.journalTitle Molecular Genetics and Metabolism Reports es
sedici.relation.journalVolumeAndIssue vol. 36 es


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Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0) Except where otherwise noted, this item's license is described as Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0)