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The Role of Oxidative Stress and Inflammation in X-Link Adrenoleukodystrophy

X-linked adrenoleukodystrophy (X-ALD) is an inherited disease caused by a mutation in the ABCD1 gene encoding a peroxisomal transmembrane protein. It is characterized by the accumulation of very-long-chain fatty acids (VLCFAs) in body fluids and tissues, leading to progressive demyelination and adre...

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Autores principales: Yu, Jiayu, Chen, Ting, Guo, Xin, Zafar, Mohammad Ishraq, Li, Huiqing, Wang, Zhihua, Zheng, Juan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9024313/
https://www.ncbi.nlm.nih.gov/pubmed/35463999
http://dx.doi.org/10.3389/fnut.2022.864358
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author Yu, Jiayu
Chen, Ting
Guo, Xin
Zafar, Mohammad Ishraq
Li, Huiqing
Wang, Zhihua
Zheng, Juan
author_facet Yu, Jiayu
Chen, Ting
Guo, Xin
Zafar, Mohammad Ishraq
Li, Huiqing
Wang, Zhihua
Zheng, Juan
author_sort Yu, Jiayu
collection PubMed
description X-linked adrenoleukodystrophy (X-ALD) is an inherited disease caused by a mutation in the ABCD1 gene encoding a peroxisomal transmembrane protein. It is characterized by the accumulation of very-long-chain fatty acids (VLCFAs) in body fluids and tissues, leading to progressive demyelination and adrenal insufficiency. ALD has various phenotypes, among which the most common and severe is childhood cerebral adrenoleukodystrophy (CCALD). The pathophysiological mechanisms of ALD remain unclear, but some in vitro/in vivo research showed that VLCFA could induce oxidative stress and inflammation, leading to damage. In addition, the evidence that oxidative stress and inflammation are increased in patients with X-ALD also proves that it is a potential mechanism of brain and adrenal damage. Therefore, normalizing the redox balance becomes a critical therapeutic target. This study focuses on the possible predictors of the severity and progression of X-ALD, the potential mechanisms of pathogenesis, and the promising targeted drugs involved in oxidative stress and inflammation.
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spelling pubmed-90243132022-04-23 The Role of Oxidative Stress and Inflammation in X-Link Adrenoleukodystrophy Yu, Jiayu Chen, Ting Guo, Xin Zafar, Mohammad Ishraq Li, Huiqing Wang, Zhihua Zheng, Juan Front Nutr Nutrition X-linked adrenoleukodystrophy (X-ALD) is an inherited disease caused by a mutation in the ABCD1 gene encoding a peroxisomal transmembrane protein. It is characterized by the accumulation of very-long-chain fatty acids (VLCFAs) in body fluids and tissues, leading to progressive demyelination and adrenal insufficiency. ALD has various phenotypes, among which the most common and severe is childhood cerebral adrenoleukodystrophy (CCALD). The pathophysiological mechanisms of ALD remain unclear, but some in vitro/in vivo research showed that VLCFA could induce oxidative stress and inflammation, leading to damage. In addition, the evidence that oxidative stress and inflammation are increased in patients with X-ALD also proves that it is a potential mechanism of brain and adrenal damage. Therefore, normalizing the redox balance becomes a critical therapeutic target. This study focuses on the possible predictors of the severity and progression of X-ALD, the potential mechanisms of pathogenesis, and the promising targeted drugs involved in oxidative stress and inflammation. Frontiers Media S.A. 2022-04-08 /pmc/articles/PMC9024313/ /pubmed/35463999 http://dx.doi.org/10.3389/fnut.2022.864358 Text en Copyright © 2022 Yu, Chen, Guo, Zafar, Li, Wang and Zheng. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Nutrition
Yu, Jiayu
Chen, Ting
Guo, Xin
Zafar, Mohammad Ishraq
Li, Huiqing
Wang, Zhihua
Zheng, Juan
The Role of Oxidative Stress and Inflammation in X-Link Adrenoleukodystrophy
title The Role of Oxidative Stress and Inflammation in X-Link Adrenoleukodystrophy
title_full The Role of Oxidative Stress and Inflammation in X-Link Adrenoleukodystrophy
title_fullStr The Role of Oxidative Stress and Inflammation in X-Link Adrenoleukodystrophy
title_full_unstemmed The Role of Oxidative Stress and Inflammation in X-Link Adrenoleukodystrophy
title_short The Role of Oxidative Stress and Inflammation in X-Link Adrenoleukodystrophy
title_sort role of oxidative stress and inflammation in x-link adrenoleukodystrophy
topic Nutrition
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9024313/
https://www.ncbi.nlm.nih.gov/pubmed/35463999
http://dx.doi.org/10.3389/fnut.2022.864358
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