Chung Ki Wha, Kim Ju Seong, Lee Kyung Suk
Department of Biological Sciences, Kongju National University, Gongju 32588, Korea.
Department of Physics Education, Kongju National University, Gongju 32588, Korea.
Mol Cells. 2020 Oct 31;43(10):880-888. doi: 10.14348/molcells.2020.0178.
Inherited peripheral neuropathy is a heterogeneous group of peripheral neurodegenerative disorders including Charcot-Marie-Tooth disease. Many peripheral neuropathies often accompany impaired axonal construction and function. To study the molecular and cellular basis of axon-defective peripheral neuropathy, we explore the possibility of using , a powerful nematode model equipped with a variety of genetics and imaging tools. In search of potential candidates of peripheral neuropathy models, we monitored the movement and the body posture patterns of 26 strains with disruption of genes associated with various peripheral neuropathies and compiled a database of their phenotypes. Our assay showed that movement features of the worms with mutations in , and human homologues are significantly different from the control strain, suggesting they are viable candidates for peripheral neuropathy models.
遗传性周围神经病是一组异质性的周围神经退行性疾病,包括夏科-马里-图斯病。许多周围神经病常伴有轴突结构和功能受损。为了研究轴突缺陷性周围神经病的分子和细胞基础,我们探索了使用一种配备多种遗传学和成像工具的强大线虫模型的可能性。为了寻找周围神经病模型的潜在候选者,我们监测了26种与各种周围神经病相关基因被破坏的菌株的运动和身体姿势模式,并编制了它们的表型数据库。我们的分析表明,与人类同源基因发生突变的蠕虫的运动特征与对照菌株有显著差异,表明它们是周围神经病模型的可行候选者。