McCombe P A, Pfluger C, Singh P, Lim C Y H, Airey C, Henderson R D
The University of Queensland, UQ Centre for Clinical Research, Royal Brisbane Hospital, Herston, Queensland 4029, Australia.
The University of Queensland, UQ Centre for Clinical Research, Royal Brisbane Hospital, Herston, Queensland 4029, Australia.
J Neurol Sci. 2015;353(1-2):122-9. doi: 10.1016/j.jns.2015.04.032. Epub 2015 Apr 29.
There is a need for a blood biomarker of disease activity in ALS. This marker needs to measure the loss of motor neurones. Phosphorylated neurofilament heavy chain (pNfH) in the serum is a biomarker of axonal injury. Previous studies have found that levels of pNfH are elevated in ALS. We have performed a serial study of pNfH levels in 98 subjects from our ALS clinic. There was significant elevation of levels of pNfH in subjects with ALS compared to controls, although there was considerable variability. In studies of individuals who had two or more serial samples, we found that the levels of pNfH increased over time in the early stage of disease. Levels were low in subjects with long survival. The rate of rise of pNfH was inversely correlated with survival. We suggest that the initial level of pNfH is a marker of disease severity and that changes in pNfH levels are markers of disease progression.
肌萎缩侧索硬化症（ALS）患者需要一种疾病活动的血液生物标志物。这种标志物需要能够测量运动神经元的损失情况。血清中的磷酸化神经丝重链（pNfH）是轴突损伤的生物标志物。先前的研究发现，ALS患者的pNfH水平会升高。我们对来自ALS诊所的98名受试者的pNfH水平进行了一项系列研究。与对照组相比，ALS患者的pNfH水平显著升高，尽管存在相当大的变异性。在对有两份或更多份连续样本的个体进行的研究中，我们发现，在疾病早期，pNfH水平随时间升高。生存期长的受试者pNfH水平较低。pNfH的升高速率与生存期呈负相关。我们认为，pNfH的初始水平是疾病严重程度的标志物，而pNfH水平的变化是疾病进展的标志物。
