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Nonhuman animal reproducing pathogenic conditions of spinal and bulbar muscular atrophy and remedy for spinal and bulbar muscular atrophy
Nonhuman animal reproducing pathogenic conditions of spinal and bulbar muscular atrophy and remedy for spinal and bulbar muscular atrophy
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机译:非人类动物繁殖脊髓和延髓肌萎缩的病原体状况以及对脊髓和延髓肌萎缩的治疗方法
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摘要
It is intended to provide a model animal faithfully reproducing the pathogenic conditions of spinal and bulbar muscular atrophy, a method of screening a remedy for polyglutamine disease using the same, and a remedy for spinal and bulbar muscular atrophy. Namely, a nonhuman animal having the following characteristics (1) to (5) in its conditions or pathological findings: (1) showing progressive myoatrophy; (2) showing lowering in muscular power; (3) in immunostaining with the use of an anti-polyglutamine antibody, showing nuclear diffuse staining and nuclear inclusions; (4) in immunostaining with the use of an anti-androgen receptor antibody, showing nuclear diffuse staining and nuclear inclusions; and (5) showing a neurogenic change. A remedy for polyglutamine disease is screened by administering a test substance to this nonhuman animal and examining changes in its conditions or pathological findings. A remedy for spinal and bulbar muscular atrophy is prepared by using as the active ingredient a compound having an effect of inhibiting the secretion of testosterone.
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