Physics Department `Sapienza' University, CNR-Institute of Nanotechnology, Piazzale Aldo Moro 5, 00185 Rome, Italy.
Department of Medical Physics and Biomedical Engineering, University College London, Gower Street, London WC1E 6BT, United Kingdom.
J Synchrotron Radiat. 2020 Jul 1;27(Pt 4):1042-1048. doi: 10.1107/S1600577520006785. Epub 2020 Jun 9.
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder affecting motor neurons. Pre-clinical studies drive the development of animal models that well mimic ALS disorder and enable both the dissection of disease processes and an early assessment of therapy efficacy. A comprehensive knowledge of neuronal and vascular lesions in the brain and spinal cord is an essential factor to understand the development of the disease. Spatial resolution and bidimensional imaging are important drawbacks limiting current neuroimaging tools, while neuropathology relies on protocols that may alter tissue chemistry and structure. In contrast, recent ex vivo studies in mice demonstrated that X-ray phase-contrast tomography enables study of the 3D distribution of both vasculature and neuronal networks, without sample sectioning or use of staining. Here we present our findings on ex vivo SOD1 ALS mice spinal cord at a micrometric scale. An unprecedented direct quantification of neuro-vascular alterations at different stages of the disease is shown.
肌萎缩侧索硬化症（ALS）是一种影响运动神经元的进行性神经退行性疾病。临床前研究推动了动物模型的发展，这些模型很好地模拟了 ALS 疾病，并能够对疾病过程进行剖析，以及早期评估治疗效果。全面了解大脑和脊髓中的神经元和血管损伤是理解疾病发展的一个重要因素。空间分辨率和二维成像都是限制当前神经影像学工具的重要缺陷，而神经病理学则依赖于可能改变组织化学和结构的方案。相比之下，最近在小鼠中的离体研究表明，X 射线相衬断层摄影术能够在不进行样本切片或使用染色的情况下，研究血管和神经元网络的三维分布。在这里，我们展示了在微尺度下对 SOD1 ALS 小鼠脊髓进行离体研究的结果。在疾病的不同阶段，对神经血管变化进行了前所未有的直接定量。
