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KMID : 0385020020180030137
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Korean Journal of Laboratory Animal Science
2002 Volume.18 No. 3 p.137 ~ p.142
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ENU (N-ethyl-N-nitrosourea)-induced Hereditary Myotonia Mouse with General Weakness
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Cho, Jae-Woo
Cho, Kyu-hyuk/Lim, Chae-Woong/Han, Sang-Seop/Song, Chang-Woo
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Abstract
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Myotonia and general weakness mutants were observed from N-ethyl-N-nitrosourea (ENU) mutagenesis. This study was carried out to identify phenotype through behavioral test and pathological investigation, and to check a genetic mode with mating experiment. This mutant mouse showed myotonia by physical stimulation from 2~3 weeks of age, and were distinguished from normal littermates. They exhibited stiff extension posture under free fall test (the height of 20㎝). Abnormal animals weighed about 35% less than normal animals at 10 weeks of age. There were no evidences of degeneration and necrosis in muscle tissues in H&E stain, but the mean diameter of gastrocnemius muscle was generally reduced. Behavioral test explained that myotonia symptom became progressive especially between 13 months of age. The segregation ration of the normal and the mutant was close to 3:1 and the numbers of males and females in mutant mice were 30 to 32. Therefore, it was determined that affected gene was transferred to the next generation by autosomal single recessive mode. In conclusion, this mutant mouse, which exhibited behavioral abnormality caused by myotonia without muscular degeneration and necrosis, may provide a model system to study human myotonia disease.
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