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Glycoprotein Non-Metastatic Protein B: An Emerging Biomarker for Lysosomal Dysfunction in Macrophages
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...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6337583/ https://www.ncbi.nlm.nih.gov/pubmed/30586924 http://dx.doi.org/10.3390/ijms20010066 |
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author | van der Lienden, Martijn J.C. Gaspar, Paulo Boot, Rolf Aerts, Johannes M.F.G. van Eijk, Marco |
author_facet | van der Lienden, Martijn J.C. Gaspar, Paulo Boot, Rolf Aerts, Johannes M.F.G. van Eijk, Marco |
author_sort | van der Lienden, Martijn J.C. |
collection | PubMed |
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. |
format | Online Article Text |
id | pubmed-6337583 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-63375832019-01-22 Glycoprotein Non-Metastatic Protein B: An Emerging Biomarker for Lysosomal Dysfunction in Macrophages van der Lienden, Martijn J.C. Gaspar, Paulo Boot, Rolf Aerts, Johannes M.F.G. van Eijk, Marco Int J Mol Sci Review 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. MDPI 2018-12-24 /pmc/articles/PMC6337583/ /pubmed/30586924 http://dx.doi.org/10.3390/ijms20010066 Text en © 2018 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review van der Lienden, Martijn J.C. Gaspar, Paulo Boot, Rolf Aerts, Johannes M.F.G. van Eijk, Marco 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 |
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_short | 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 |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6337583/ https://www.ncbi.nlm.nih.gov/pubmed/30586924 http://dx.doi.org/10.3390/ijms20010066 |
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