Glycoprotein Non-Metastatic Protein B: an Emerging Biomarker for Lysosomal Dysfunction in Macrophages

Detalhes bibliográficos
Autor(a) principal: van der Lienden, Martijn
Data de Publicação: 2018
Outros Autores: Gaspar, Paulo, Boot, Rolf, Aerts, Johannes, van Eijk, Marco
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://hdl.handle.net/10400.18/6262
Resumo: Several diseases are caused by inherited defects in lysosomes, the so-called lysosomal storage disorders (LSDs). In some of these LSDs, tissue macrophages transform into prominent storage cells, as is the case in Gaucher disease. Here, macrophages become the characteristic Gaucher cells filled with lysosomes laden with glucosylceramide, because of their impaired enzymatic degradation. Biomarkers of Gaucher cells were actively searched, particularly after the development of costly therapies based on enzyme supplementation and substrate reduction. Proteins selectively expressed by storage macrophages and secreted into the circulation were identified, among which glycoprotein non-metastatic protein B (GPNMB). This review focusses on the emerging potential of GPNMB as a biomarker of stressed macrophages in LSDs as well as in acquired pathologies accompanied by an excessive lysosomal substrate load in macrophages.
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spelling Glycoprotein Non-Metastatic Protein B: an Emerging Biomarker for Lysosomal Dysfunction in MacrophagesGPNMBMITFAutophagyFoam CellInflammationLysosomal Storage DisordersLysosomeMacrophageMetabolic SyndromePhagocytosisDoenças GenéticasSeveral diseases are caused by inherited defects in lysosomes, the so-called lysosomal storage disorders (LSDs). In some of these LSDs, tissue macrophages transform into prominent storage cells, as is the case in Gaucher disease. Here, macrophages become the characteristic Gaucher cells filled with lysosomes laden with glucosylceramide, because of their impaired enzymatic degradation. Biomarkers of Gaucher cells were actively searched, particularly after the development of costly therapies based on enzyme supplementation and substrate reduction. Proteins selectively expressed by storage macrophages and secreted into the circulation were identified, among which glycoprotein non-metastatic protein B (GPNMB). This review focusses on the emerging potential of GPNMB as a biomarker of stressed macrophages in LSDs as well as in acquired pathologies accompanied by an excessive lysosomal substrate load in macrophages.This work was supported by the Dutch Diabetes Foundation (2014.00.1758).MDPIRepositório Científico do Instituto Nacional de Saúdevan der Lienden, MartijnGaspar, PauloBoot, RolfAerts, Johannesvan Eijk, Marco2019-03-21T12:36:18Z2018-12-242018-12-24T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10400.18/6262engInt J Mol Sci. 2018 Dec 24;20(1). pii: E66. doi: 10.3390/ijms200100661661-659610.3390/ijms20010066info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2023-07-20T15:41:19Zoai:repositorio.insa.pt:10400.18/6262Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T18:40:55.535335Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv Glycoprotein Non-Metastatic Protein B: an Emerging Biomarker for Lysosomal Dysfunction in Macrophages
title Glycoprotein Non-Metastatic Protein B: an Emerging Biomarker for Lysosomal Dysfunction in Macrophages
spellingShingle Glycoprotein Non-Metastatic Protein B: an Emerging Biomarker for Lysosomal Dysfunction in Macrophages
van der Lienden, Martijn
GPNMB
MITF
Autophagy
Foam Cell
Inflammation
Lysosomal Storage Disorders
Lysosome
Macrophage
Metabolic Syndrome
Phagocytosis
Doenças Genéticas
title_short Glycoprotein Non-Metastatic Protein B: an Emerging Biomarker for Lysosomal Dysfunction in Macrophages
title_full Glycoprotein Non-Metastatic Protein B: an Emerging Biomarker for Lysosomal Dysfunction in Macrophages
title_fullStr Glycoprotein Non-Metastatic Protein B: an Emerging Biomarker for Lysosomal Dysfunction in Macrophages
title_full_unstemmed Glycoprotein Non-Metastatic Protein B: an Emerging Biomarker for Lysosomal Dysfunction in Macrophages
title_sort Glycoprotein Non-Metastatic Protein B: an Emerging Biomarker for Lysosomal Dysfunction in Macrophages
author van der Lienden, Martijn
author_facet van der Lienden, Martijn
Gaspar, Paulo
Boot, Rolf
Aerts, Johannes
van Eijk, Marco
author_role author
author2 Gaspar, Paulo
Boot, Rolf
Aerts, Johannes
van Eijk, Marco
author2_role author
author
author
author
dc.contributor.none.fl_str_mv Repositório Científico do Instituto Nacional de Saúde
dc.contributor.author.fl_str_mv van der Lienden, Martijn
Gaspar, Paulo
Boot, Rolf
Aerts, Johannes
van Eijk, Marco
dc.subject.por.fl_str_mv GPNMB
MITF
Autophagy
Foam Cell
Inflammation
Lysosomal Storage Disorders
Lysosome
Macrophage
Metabolic Syndrome
Phagocytosis
Doenças Genéticas
topic GPNMB
MITF
Autophagy
Foam Cell
Inflammation
Lysosomal Storage Disorders
Lysosome
Macrophage
Metabolic Syndrome
Phagocytosis
Doenças Genéticas
description Several diseases are caused by inherited defects in lysosomes, the so-called lysosomal storage disorders (LSDs). In some of these LSDs, tissue macrophages transform into prominent storage cells, as is the case in Gaucher disease. Here, macrophages become the characteristic Gaucher cells filled with lysosomes laden with glucosylceramide, because of their impaired enzymatic degradation. Biomarkers of Gaucher cells were actively searched, particularly after the development of costly therapies based on enzyme supplementation and substrate reduction. Proteins selectively expressed by storage macrophages and secreted into the circulation were identified, among which glycoprotein non-metastatic protein B (GPNMB). This review focusses on the emerging potential of GPNMB as a biomarker of stressed macrophages in LSDs as well as in acquired pathologies accompanied by an excessive lysosomal substrate load in macrophages.
publishDate 2018
dc.date.none.fl_str_mv 2018-12-24
2018-12-24T00:00:00Z
2019-03-21T12:36:18Z
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://hdl.handle.net/10400.18/6262
url http://hdl.handle.net/10400.18/6262
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv Int J Mol Sci. 2018 Dec 24;20(1). pii: E66. doi: 10.3390/ijms20010066
1661-6596
10.3390/ijms20010066
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv MDPI
publisher.none.fl_str_mv MDPI
dc.source.none.fl_str_mv reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron:RCAAP
instname_str Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron_str RCAAP
institution RCAAP
reponame_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
collection Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository.name.fl_str_mv Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
repository.mail.fl_str_mv
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