Laboratoire Neurobiologie and Transgenese, LUNAM, UPRES EA-3143, Centre Hospitalier Universitaire, Bâtiment IBS-IRIS, Université d'Angers, 49033, Angers, France.
Histochem Cell Biol. 2013 Jul;140(1):13-22. doi: 10.1007/s00418-013-1101-1. Epub 2013 Jun 8.
Intermediate filaments (IFs) of the nervous system, including neurofilaments, α-internexin, glial fibrillary acidic protein, synemin, nestin, peripherin and vimentin, are finely expressed following elaborated cell, tissue and developmental specific patterns. A common characteristic of several neurodegenerative diseases is the abnormal accumulation of neuronal IFs in cell bodies or along the axon, often associated with impairment of the axonal transport and degeneration of neurons. In this review, we also present several perturbations of IF metabolism and organization associated with neurodegenerative disorders. Such modifications could represent strong markers of neuronal damages. Moreover, recent data suggest that IFs represent potential biomarkers to determine the disease progression or the differential stages of a neuronal disorder. Finally, recent investigations on IF expression and function in cancer provide evidence that they may be useful as markers, or targets of brain tumours, especially high-grade glioma. A better knowledge of the molecular mechanisms of IF alterations, combined to neuroimaging, is essential to improve diagnosis and therapeutic strategies of such neurodegenerative diseases and glioma.
神经系统中间丝（IFs），包括神经丝、α-中间丝结合蛋白、胶质纤维酸性蛋白、突触素、巢蛋白、外周蛋白和波形蛋白，在经过精心设计的细胞、组织和发育特异性模式后精细表达。几种神经退行性疾病的一个共同特征是神经元 IF 在细胞体或沿着轴突异常积累，通常与轴突运输受损和神经元变性有关。在这篇综述中，我们还介绍了与神经退行性疾病相关的几种中间丝代谢和组织的改变。这些改变可能是神经元损伤的强标志物。此外，最近的数据表明，IFs 可能是确定疾病进展或神经元疾病的不同阶段的潜在生物标志物。最后，最近关于 IF 在癌症中的表达和功能的研究提供了证据，表明它们可能作为标志物或脑肿瘤的靶点，特别是高级别神经胶质瘤。更好地了解 IF 改变的分子机制，结合神经影像学，可以提高这些神经退行性疾病和神经胶质瘤的诊断和治疗策略。
