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威尔逊病的生物标志物发现——通往改善诊断和管理之路:全面综述

Biomarker Discovery in Wilson's Disease-A Path Toward Improved Diagnosis and Management: A Comprehensive Review.

作者信息

Wolska Marta, Wicik Zofia, Ahmadova Sara, Mirowska-Guzel Dagmara, Postula Marek, Czlonkowska Anna, Eyileten Ceren

机构信息

Department of Experimental and Clinical Pharmacology, Center for Preclinical Research and Technology CEPT, Medical University of Warsaw, Banacha 1B Str, 02-097, Warsaw, Poland.

Doctoral School, Medical University of Warsaw, Warsaw, Poland.

出版信息

Mol Neurobiol. 2025 Jun 18. doi: 10.1007/s12035-025-05143-6.

Abstract

Wilson's disease (WD) is a rare autosomal recessive disorder characterized by defective copper metabolism, which leads to hepatic and neurological damage. The clinical presentation of WD varies significantly, often resulting in delayed diagnosis and an increased risk of irreversible complications. Current diagnostic tools, including biochemical assays, imaging techniques, and genetic testing, lack sufficient specificity and sensitivity, highlighting the need for novel biomarkers for early diagnosis and treatment monitoring. This review explores emerging biomarkers for both hepatic and neurological manifestations of WD, including blood-based molecular markers such as cytokines, proteases, oxidative stress indicators, inflammasomes, and gut microbiota signatures. Recent studies have identified neurofilament light chain (NfL), pentraxin 3 (PTX3), caspase-3/XIAP, and NLRP3 inflammasome activation as promising indicators of neurological impairment. Additionally, markers like soluble CD163 (sCD163) and apoptosis antigen 1 (APO-1) show potential for assessing hepatic dysfunction. Metabolomic and proteomic analyses further suggest distinct molecular profiles associated with different WD subtypes, while microRNA-based biomarkers offer novel insights into disease progression. Identifying and validating these biomarkers could enhance early diagnosis, predict neurological deterioration, and optimize treatment strategies, ultimately improving patient outcomes. Further research is needed to integrate these biomarkers into clinical practice and establish standardized protocols for their use in WD management.

摘要

威尔逊病(WD)是一种罕见的常染色体隐性疾病,其特征为铜代谢缺陷,可导致肝脏和神经损伤。WD的临床表现差异很大,常常导致诊断延迟以及不可逆并发症风险增加。目前的诊断工具,包括生化检测、成像技术和基因检测,缺乏足够的特异性和敏感性,这凸显了需要新的生物标志物用于早期诊断和治疗监测。本综述探讨了WD肝脏和神经表现的新兴生物标志物,包括基于血液的分子标志物,如细胞因子、蛋白酶、氧化应激指标、炎性小体和肠道微生物群特征。最近的研究已确定神经丝轻链(NfL)、五聚体3(PTX3)、半胱天冬酶-3/ X连锁凋亡抑制蛋白(XIAP)和NLRP3炎性小体激活是神经损伤的有前景指标。此外,可溶性CD163(sCD163)和凋亡抗原1(APO-1)等标志物显示出评估肝功能障碍的潜力。代谢组学和蛋白质组学分析进一步表明与不同WD亚型相关的独特分子谱,而基于微小RNA的生物标志物为疾病进展提供了新的见解。识别和验证这些生物标志物可加强早期诊断、预测神经恶化并优化治疗策略,最终改善患者预后。需要进一步研究将这些生物标志物整合到临床实践中,并建立在WD管理中使用它们的标准化方案。

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