从基因到疗法:解读 ABCD1 在防治 X-连锁肾上腺白质营养不良症中的作用综述。

IF 3.9 2区 医学 Q2 BIOCHEMISTRY & MOLECULAR BIOLOGY
Xinxin Zuo, Zeyu Chen
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引用次数: 0

摘要

X连锁肾上腺白质营养不良症(X-ALD)是一种严重的遗传性疾病,由ABCD1基因突变引起,会导致超长链脂肪酸积聚,导致神经系统功能衰退和肾上腺功能不全。尽管人们对 X-ALD 的发病机制有了更深入的了解,但对其病理生理学的认识仍不全面,这使得开发有效治疗方法变得更加复杂。本综述全面概述了 X-ALD 的情况,重点关注 ABCD1 的遗传和生化作用及其突变的影响。本综述对目前的治疗方法进行了评估,讨论了这些方法的局限性,并强调了全面阐明 X-ALD 发病机制的必要性。此外,这篇综述还强调了国际合作对加强系统数据收集和推进生物标志物发现的重要性,最终改善 X-ALD 患者的预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
From gene to therapy: a review of deciphering the role of ABCD1 in combating X-Linked adrenoleukodystrophy.

X-linked adrenoleukodystrophy (X-ALD) is a severe genetic disorder caused by ABCD1 mutations, resulting in the buildup of very-long-chain fatty acids, leading to significant neurological decline and adrenal insufficiency. Despite advancements in understanding the mechanisms of X-ALD, its pathophysiology remains incompletely understood, complicating the development of effective treatments. This review provides a comprehensive overview of X-ALD, with a focus on the genetic and biochemical roles of ABCD1 and the impacts of its mutations. Current therapeutic approaches are evaluated, discussing their limitations, and emphasizing the need to fully elucidate the pathogenesis of X-ALD. Additionally, this review highlights the importance of international collaboration to enhance systematic data collection and advance biomarker discovery, ultimately improving patient outcomes with X-ALD.

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来源期刊
Lipids in Health and Disease
Lipids in Health and Disease 生物-生化与分子生物学
CiteScore
7.70
自引率
2.20%
发文量
122
审稿时长
3-8 weeks
期刊介绍: Lipids in Health and Disease is an open access, peer-reviewed, journal that publishes articles on all aspects of lipids: their biochemistry, pharmacology, toxicology, role in health and disease, and the synthesis of new lipid compounds. Lipids in Health and Disease is aimed at all scientists, health professionals and physicians interested in the area of lipids. Lipids are defined here in their broadest sense, to include: cholesterol, essential fatty acids, saturated fatty acids, phospholipids, inositol lipids, second messenger lipids, enzymes and synthetic machinery that is involved in the metabolism of various lipids in the cells and tissues, and also various aspects of lipid transport, etc. In addition, the journal also publishes research that investigates and defines the role of lipids in various physiological processes, pathology and disease. In particular, the journal aims to bridge the gap between the bench and the clinic by publishing articles that are particularly relevant to human diseases and the role of lipids in the management of various diseases.
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