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Treatment of Fabry Disease: Established and Emerging Therapies
Fabry disease (FD) is a rare, X-linked inherited disorder of glycosphingolipid metabolism. It leads to the progressive accumulation of globotriaosylceramide within lysosomes due to a deficiency of α-galactosidase A enzyme. It involves multiple organs, predominantly the renal, cardiac, and cerebrovas...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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MDPI
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9967779/ https://www.ncbi.nlm.nih.gov/pubmed/37259462 http://dx.doi.org/10.3390/ph16020320 |
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author | Umer, Muhammad Kalra, Dinesh K. |
author_facet | Umer, Muhammad Kalra, Dinesh K. |
author_sort | Umer, Muhammad |
collection | PubMed |
description | Fabry disease (FD) is a rare, X-linked inherited disorder of glycosphingolipid metabolism. It leads to the progressive accumulation of globotriaosylceramide within lysosomes due to a deficiency of α-galactosidase A enzyme. It involves multiple organs, predominantly the renal, cardiac, and cerebrovascular systems. Early diagnosis and treatment are critical to prevent progression to irreversible tissue damage and organ failure, and to halt life-threatening complications that can significantly reduce life expectancy. This review will focus on the established and emerging treatment options for FD. |
format | Online Article Text |
id | pubmed-9967779 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-99677792023-02-27 Treatment of Fabry Disease: Established and Emerging Therapies Umer, Muhammad Kalra, Dinesh K. Pharmaceuticals (Basel) Review Fabry disease (FD) is a rare, X-linked inherited disorder of glycosphingolipid metabolism. It leads to the progressive accumulation of globotriaosylceramide within lysosomes due to a deficiency of α-galactosidase A enzyme. It involves multiple organs, predominantly the renal, cardiac, and cerebrovascular systems. Early diagnosis and treatment are critical to prevent progression to irreversible tissue damage and organ failure, and to halt life-threatening complications that can significantly reduce life expectancy. This review will focus on the established and emerging treatment options for FD. MDPI 2023-02-20 /pmc/articles/PMC9967779/ /pubmed/37259462 http://dx.doi.org/10.3390/ph16020320 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/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 (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Umer, Muhammad Kalra, Dinesh K. Treatment of Fabry Disease: Established and Emerging Therapies |
title | Treatment of Fabry Disease: Established and Emerging Therapies |
title_full | Treatment of Fabry Disease: Established and Emerging Therapies |
title_fullStr | Treatment of Fabry Disease: Established and Emerging Therapies |
title_full_unstemmed | Treatment of Fabry Disease: Established and Emerging Therapies |
title_short | Treatment of Fabry Disease: Established and Emerging Therapies |
title_sort | treatment of fabry disease: established and emerging therapies |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9967779/ https://www.ncbi.nlm.nih.gov/pubmed/37259462 http://dx.doi.org/10.3390/ph16020320 |
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