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1. 发明申请

US20100279402A1 Expression Construct for Digesting Aggregating Protein and Method of Inhibiting the Aggregation of Aggregating Protein 审中-公开
标题翻译：用于消化聚集蛋白的表达构建体和抑制聚集蛋白聚集的方法
公开(公告)号：US20100279402A1
公开(公告)日：2010-11-04
申请号：US12225311
申请日：2007-03-19
申请人： Shin-ichi Yamada , Jyun-ichi Niwa , Gen Sobue
发明人： Shin-ichi Yamada , Jyun-ichi Niwa , Gen Sobue
IPC分类号： C12N5/071 , C12N15/79 , C12N5/07
CPC分类号： C12N9/52 , C12N15/85
摘要： It is intended to provide a means which is efficacious in digesting a protein forming aggregates in a eukaryotic cell such as mutant superoxide dismutase 1 or an androgen receptor having an abnormally extended polyglutamine chain. Namely, an expression construct for digesting an aggregating protein which has a nucleic acid encoding an archaeal proteasome and being connected in an operable manner to a promoter for eukaryotic cells. By transferring this expression construct into a eukaryotic cell, the aggregating protein is digested owing to the action of the archaeal proteasome.
摘要翻译：旨在提供一种在真核细胞如突变型超氧化物歧化酶1或具有异常延长的聚谷氨酰胺链的雄激素受体中消化形成聚集体的蛋白质有效的方法。即，用于消化具有编码古细菌蛋白酶体的核酸并以可操作的方式连接到真核细胞的启动子的聚集蛋白的表达构建体。通过将该表达构建体转移到真核细胞中，由于古细菌蛋白酶体的作用，聚集蛋白被消化。

2. 发明申请

US20080182775A1 Nonhuman animal reproducing pathogenic conditions of spinal and bulbar muscular atrophy and remedy for spinal and bulbar muscular atrophy 有权
标题翻译：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
申请人： Gen Sobue , Masahisa Katsuno , Hiroaki Adachi
发明人： Gen Sobue , Masahisa Katsuno , Hiroaki Adachi
IPC分类号： A61K38/22 , A61P21/00
CPC分类号： C12N15/8509 , A01K67/0275 , A01K2217/05 , A01K2227/105 , A01K2267/0306
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。

3. 发明授权

US07727952B2 Methods for treating spinal and bulbar muscular atrophy using LHRH analogs 有权
标题翻译：使用LHRH类似物治疗脊髓和延髓肌肉萎缩的方法
公开(公告)号：US07727952B2
公开(公告)日：2010-06-01
申请号：US11518209
申请日：2006-09-11
申请人： Gen Sobue , Masahisa Katsuno , Hiroaki Adachi
发明人： Gen Sobue , Masahisa Katsuno , Hiroaki Adachi
IPC分类号： A61K38/09 , A61K38/24
CPC分类号： C12N15/8509 , A01K67/0275 , A01K2217/05 , A01K2227/105 , A01K2267/0306
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。

4. 发明申请

US20060064767A1 Nonhuman animal reproducing pathogenic conditions of spinal and bulbar muscular atrophy and remedy for spinal and bulbar muscular atrophy 审中-公开
标题翻译：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：US20060064767A1
公开(公告)日：2006-03-23
申请号：US10524823
申请日：2003-08-18
申请人： Gen Sobue , Masahisa Katsuno , Hiroaki Adachi
发明人： Gen Sobue , Masahisa Katsuno , Hiroaki Adachi
IPC分类号： A01K67/027
CPC分类号： C12N15/8509 , A01K67/0275 , A01K2217/05 , A01K2227/105 , A01K2267/0306
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。

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