Department of Neurosciences, University of California San Diego, 9500 Gilman Drive, La Jolla, CA, 92093, USA.
Department of Medicine, University of California San Diego, 9500 Gilman Drive, La Jolla, CA, 92093, USA.
Lipids Health Dis. 2024 Nov 11;23(1):369. doi: 10.1186/s12944-024-02361-0.
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.
X 连锁肾上腺脑白质营养不良(X-ALD)是一种由 ABCD1 突变引起的严重遗传性疾病,导致极长链脂肪酸的堆积,从而导致严重的神经功能下降和肾上腺功能不全。尽管人们对 X-ALD 的发病机制有了更多的了解,但它的病理生理学仍不完全清楚,这使得有效的治疗方法难以开发。这篇综述全面概述了 X-ALD,重点介绍了 ABCD1 的遗传和生化作用及其突变的影响。评估了当前的治疗方法,讨论了它们的局限性,并强调了需要充分阐明 X-ALD 的发病机制。此外,该综述还强调了国际合作的重要性,以加强系统数据收集和推进生物标志物的发现,最终改善 X-ALD 患者的预后。