研究脊髓发育和疾病的神经肌肉器官模型

Q4 Biochemistry, Genetics and Molecular Biology
Tobias Grass, Zeynep Dokuzluoglu, Natalia Rodríguez-Muela
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引用次数: 0

摘要

神经退行性疾病病理学的许多方面仍未解决。为什么某些神经元亚群容易受到压力或普遍表达基因突变的影响,而另一些神经元亚群却仍有生命力?这些神经元是否蕴藏着使其容易发生变性的内在标记?这些疾病是否与神经发育有关?缺乏这些基础知识阻碍了有效治疗方法的发现。虽然人类类器官模型可用于大脑相关疾病的建模已得到公认,但我们仍然缺乏一种能再现脊髓区域化复杂性和生理学的类器官模型。在这里,我们描述了一种先进的实验方案,它能生成由脊髓运动神经元(spMNs)和中胚层祖细胞衍生的肌肉细胞组成的神经肌肉类器官。因此,该模型可对神经肌肉单位的发育和疾病进行稳健且可重复的研究。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Neuromuscular Organoids to Study Spinal Cord Development and Disease.

Many aspects of neurodegenerative disease pathology remain unresolved. Why do certain neuronal subpopulations acquire vulnerability to stress or mutations in ubiquitously expressed genes, while others remain resilient? Do these neurons harbor intrinsic marks that make them prone to degeneration? Do these diseases have a neurodevelopmental component? Lacking this fundamental knowledge hampers the discovery of efficacious treatments. While it is well established that human organoids enable the modeling of brain-related diseases, we still lack an organoid model that recapitulates the regionalization complexity and physiology of the spinal cord. Here, we describe an advanced experimental protocol to generate neuromuscular organoids composed of a wide rostro-caudal (RC) diversity of spinal motor neurons (spMNs) and mesodermal progenitor-derived muscle cells. This model therefore allows for the robust and reproducible study of neuromuscular unit development and disease.

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来源期刊
Methods in molecular biology
Methods in molecular biology Biochemistry, Genetics and Molecular Biology-Genetics
CiteScore
2.00
自引率
0.00%
发文量
3536
期刊介绍: For over 20 years, biological scientists have come to rely on the research protocols and methodologies in the critically acclaimed Methods in Molecular Biology series. The series was the first to introduce the step-by-step protocols approach that has become the standard in all biomedical protocol publishing. Each protocol is provided in readily-reproducible step-by-step fashion, opening with an introductory overview, a list of the materials and reagents needed to complete the experiment, and followed by a detailed procedure that is supported with a helpful notes section offering tips and tricks of the trade as well as troubleshooting advice.
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