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Are Antioxidants a Potential Therapy for FSHD? A Review of the Literature

Facioscapulohumeral muscular dystrophy (FSHD) is an inherited myopathy affecting approximately 1 in 7500 individuals worldwide. It is a progressive disease characterised by skeletal muscle weakness and wasting. A genetic mutation on the 4q35 chromosome results in the expression of the double homeobo...

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Autores principales: Denny, Adam Philip, Heather, Alison Kay
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5485364/
https://www.ncbi.nlm.nih.gov/pubmed/28690764
http://dx.doi.org/10.1155/2017/7020295
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author Denny, Adam Philip
Heather, Alison Kay
author_facet Denny, Adam Philip
Heather, Alison Kay
author_sort Denny, Adam Philip
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description Facioscapulohumeral muscular dystrophy (FSHD) is an inherited myopathy affecting approximately 1 in 7500 individuals worldwide. It is a progressive disease characterised by skeletal muscle weakness and wasting. A genetic mutation on the 4q35 chromosome results in the expression of the double homeobox 4 gene (DUX4) which drives oxidative stress, inflammation, toxicity, and atrophy within the skeletal muscle. FSHD is characterised by oxidative stress, and there is currently no cure and a lack of therapies for the disease. Antioxidants have been researched for many years, with investigators aiming to use antioxidants therapeutically for oxidative stress-associated diseases. This has included both natural and synthetic antioxidants. The use of antioxidants in preclinical or clinical models has been largely successful with a plethora of research reporting positive results. However, when translated to clinical trials, the use of antioxidants as a therapeutic intervention for a variety of disease has been largely unsuccessful. Moreover, specifically focusing on FSHD, limited research has been conducted on the use of antioxidants as a therapy in either preclinical or clinical models. This review summarises the current state of antioxidant use in the treatment of FSHD and discusses their potential avenue for therapeutic use for FSHD patients.
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spelling pubmed-54853642017-07-09 Are Antioxidants a Potential Therapy for FSHD? A Review of the Literature Denny, Adam Philip Heather, Alison Kay Oxid Med Cell Longev Review Article Facioscapulohumeral muscular dystrophy (FSHD) is an inherited myopathy affecting approximately 1 in 7500 individuals worldwide. It is a progressive disease characterised by skeletal muscle weakness and wasting. A genetic mutation on the 4q35 chromosome results in the expression of the double homeobox 4 gene (DUX4) which drives oxidative stress, inflammation, toxicity, and atrophy within the skeletal muscle. FSHD is characterised by oxidative stress, and there is currently no cure and a lack of therapies for the disease. Antioxidants have been researched for many years, with investigators aiming to use antioxidants therapeutically for oxidative stress-associated diseases. This has included both natural and synthetic antioxidants. The use of antioxidants in preclinical or clinical models has been largely successful with a plethora of research reporting positive results. However, when translated to clinical trials, the use of antioxidants as a therapeutic intervention for a variety of disease has been largely unsuccessful. Moreover, specifically focusing on FSHD, limited research has been conducted on the use of antioxidants as a therapy in either preclinical or clinical models. This review summarises the current state of antioxidant use in the treatment of FSHD and discusses their potential avenue for therapeutic use for FSHD patients. Hindawi 2017 2017-06-12 /pmc/articles/PMC5485364/ /pubmed/28690764 http://dx.doi.org/10.1155/2017/7020295 Text en Copyright © 2017 Adam Philip Denny and Alison Kay Heather. http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review Article
Denny, Adam Philip
Heather, Alison Kay
Are Antioxidants a Potential Therapy for FSHD? A Review of the Literature
title Are Antioxidants a Potential Therapy for FSHD? A Review of the Literature
title_full Are Antioxidants a Potential Therapy for FSHD? A Review of the Literature
title_fullStr Are Antioxidants a Potential Therapy for FSHD? A Review of the Literature
title_full_unstemmed Are Antioxidants a Potential Therapy for FSHD? A Review of the Literature
title_short Are Antioxidants a Potential Therapy for FSHD? A Review of the Literature
title_sort are antioxidants a potential therapy for fshd? a review of the literature
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5485364/
https://www.ncbi.nlm.nih.gov/pubmed/28690764
http://dx.doi.org/10.1155/2017/7020295
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