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神经纤维光链在全身性 Amyloidosis:一个系统性审查

Milou Berends¹, Hans L A Nienhuis¹, David Adams²

¹Department of Internal Medicine, Amyloidosis Center of Expertise, University Medical Center Groningen, 9700 RB Groningen, The Netherlands.

International journal of molecular sciences

|April 13, 2024

概括

神经纤维光链 (NfL) 是一种早期和敏感的血液生物标志物,用于检测系统性粉症的神经病变. NfL水平可以监测疾病的进展和治疗的有效性,帮助早期诊断.

科学领域:

神经学神经学
生物标志物生物标志物
遗传学是一个遗传学.

背景情况:

周围和自主神经病变在全身性氨基粉症中很常见.
神经纤维光链 (NfL) 是神经元特定的生物标志物,在神经元损伤时释放出来.
需要一个早期,敏感的血液生物标志物,用于系统性粉样化症中的多神经病变.

研究的目的:

审查NfL在系统性粉症中早期神经病变检测中的实用性.
评估NfL在监测中枢神经系统参与,神经病变进展和治疗反应方面的作用.
为了评估NfL作为一个生物标志物在TTRv携带者.

主要方法:

对系统性粉症和TTRv载体中NfL水平的研究进行系统性文献综述.
在PubMed,Embase和Web of Science进行的搜索截至2024年2月14日.
包括原始数据的研究,共计1604名参与者 (298个对照组,1306名TRv携带者/患者).

主要成果:

与对照组和无症状携带者相比,在多神经病症患者中观察到较高的NfL水平.
增加的NfL水平与疾病发作相关.
在启动过敏激素基因沉默剂治疗后,NfL水平下降,并保持稳定.

结论:

NfL作为一个早期和敏感的疾病过程生物标志物用于神经病变在全身性粉症.
NfL不是一个结果生物标志物.
NfL显示了早期神经病变检测,治疗效果监测和疾病进展的潜力.

关键词:

自主神经病变自主神经病变生物标志物生物标志物遗传性转基因氨基基胺.免疫球体蛋白轻链粉样蛋白神经纤维光链的光链.多性神经病症多性神经病症.小纤维神经病变小纤维神经病变系统性氨基粉症.转胺基因变异载体基因变异

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