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Advances in the Research of Human Charcot-Marie-Tooth II Disease Using the Drosophila


YU Lingqi, XIE Jiayu, YUAN Yao, LING Sheng’an, CHEN Wenfeng*

(College of Biological Science and Engineering, Fuzhou University, Fuzhou 350108, China)
Abstract:

CMT (Charcot-Marie-Tooth) is a common family-genetic peripheral nervous system disease usually caused by mutations in certain protein defects in neurons, which are characterized by defects in distal sensory and motor neurons. CMT patients show insufficient mobility and severe cases can lose mobility. According to clinical and electrophysiological characteristics, CMT is mainly divided into primary demyelinating lesions CMT1, primary axonal lesion CMT2, secondary demyelinating and axonal lesions DI-CMT. More and more researches are using the Drosophila models to simulate various aspects of human disease and human health related processes. Drosophila has no axons surrounded by myelin, so it is not suitable for establishing a demyelinating CMT model, and is more suitable for CMT2 researches. Here, this review mainly analyzes CMT2, summarizing the related pathogenic genes involved in human CMT2, and describes how to use the Drosophila for CMT2 model construction and pathological analysis. This will be of great significance for the biological and medical researches of CMT2 diseases


CSTR: 32200.14.cjcb.2023.01.0014