关键词: Amyotrophic lateral sclerosis Axon Axotomy Cranial motoneuron Differentiation protocol Microfluidic device Spinal motoneuron iPSC piggyBac

Mesh : Amyotrophic Lateral Sclerosis Axons / physiology Humans Induced Pluripotent Stem Cells Lab-On-A-Chip Devices Motor Neurons Phenotype

来  源:   DOI:10.1007/978-1-0716-1979-7_12

Abstract:
In order to use induced Pluripotent Stem Cells (iPSCs) to model neurodegenerative diseases, efficient and homogeneous generation of neurons in vitro represents a key step. Here we describe a method to obtain and characterize functional human spinal and cranial motoneurons using a combined approach of microfluidic chips and programs designed for scientific multidimensional imaging. We have used this approach to analyze axonal phenotypes. These tools are useful to investigate the cellular and molecular bases of neuromuscular diseases, including amyotrophic lateral sclerosis and spinal muscular atrophy.
摘要:
为了使用诱导多能干细胞(iPSCs)来模拟神经退行性疾病,体外神经元的有效和均匀生成是关键步骤。在这里,我们描述了一种使用微流体芯片和设计用于科学多维成像的程序的组合方法来获得和表征功能性人类脊髓和颅运动神经元的方法。我们已经使用这种方法来分析轴突表型。这些工具可用于研究神经肌肉疾病的细胞和分子基础,包括肌萎缩侧索硬化症和脊髓性肌萎缩症。
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