Nonhuman animal reproducing pathogenic conditions of spinal and bulbar muscular atrophy and remedy for spinal and bulbar muscular atrophy
    41.
    发明申请
    Nonhuman animal reproducing pathogenic conditions of spinal and bulbar muscular atrophy and remedy for spinal and bulbar muscular atrophy 有权
    非人动物繁殖脊髓和延髓肌肉萎缩的病原性病症,脊髓和延髓肌肉萎缩的补救措施

    公开(公告)号:US20080182775A1

    公开(公告)日:2008-07-31

    申请号:US11518209

    申请日:2006-09-11

    IPC分类号: A61K38/22 A61P21/00

    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.

    摘要翻译: 旨在提供一种忠实地复制脊髓和延髓肌肉萎缩的致病条件的模型动物,使用其来筛选聚谷氨酰胺疾病的治疗方法,以及脊髓和延髓肌肉萎缩的补救措施。 即,具有以下特征(1)〜(5)的病例或病理结果的非人动物:(1)显示进行性肌萎缩; (2)显示肌肉力量的下降; (3)在使用抗多聚谷氨酰胺抗体的免疫染色中显示核扩散染色和核内含物; (4)使用抗雄激素受体抗体进行免疫染色,显示核扩散染色和核内含物; 和(5)显示神经源性变化。 通过向该非人动物施用测试物质并检查其病症或病理学发现的变化来筛选聚谷氨酰胺病的补救措施。 通过使用具有抑制睾酮分泌作用的化合物作为活性成分制备脊髓和延髓肌肉萎缩的补救措施。