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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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Detalles Bibliográficos
Autores principales: Umer, Muhammad, Kalra, Dinesh K.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
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.
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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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