Pathogenesis of cardiomyopathy
    1.
    发明授权
    Pathogenesis of cardiomyopathy 失效
    心肌病发病机制

    公开(公告)号:US06201168B1

    公开(公告)日:2001-03-13

    申请号:US09378418

    申请日:1999-08-20

    IPC分类号: A01K67027

    摘要: Disclosed is a mouse, cells derived therefrom, and methods for using the mouse, the mouse being homozygous for a disrupted &dgr;-sarcoglycan gene, the disruption in the gene having been introduced into the mouse or an ancestor of the mouse at an embryonic stage. The disruption prevents the synthesis of functional &dgr;-sarcoglycan in cells of the mouse and results in the mouse having a reduced amount of &bgr;- and &egr;-sarcoglycan and sarcospan, and a disruption of the sarcoglycan-sarcospan complex in smooth muscle of the mouse. Also disclosed is a mouse, cells derived therefrom, and methods for using the mouse, the mouse being homozygous for a disrupted &bgr;-sarcoglycan gene, the disruption in the gene having been introduced into the mouse or an ancestor of the mouse at an embryonic stage. The disruption prevents the synthesis of functional &bgr;-sarcoglycan in cells of the mouse and results in the mouse having a reduced amount of &dgr;-and &egr;-sarcoglycan and sarcospan and &agr;-dystroglycan in smooth muscle of the mouse.

    摘要翻译: 公开了一种小鼠,其衍生的细胞,以及使用该小鼠的方法,该小鼠对于破坏的δ-色氨酸聚糖基因是纯合的,该基因在胚胎阶段被导入小鼠或该小鼠祖先的破坏。 这种破坏阻止了小鼠细胞中功能性δ-糖聚糖的合成,并导致小鼠β-谷氨酸和唾液酸和焦糖的量减少,并且导致小鼠平滑肌中的slegoglycan-sarcospan复合物的破坏。 还公开了一种小鼠,由其衍生的细胞,以及使用该小鼠的方法,该小鼠对于破坏的β-唾液酸聚糖基因是纯合的,该基因在胚胎阶段被导入小鼠或小鼠祖先的破坏 。 破坏阻止了小鼠细胞中功能性β-sogoglycan的合成,并导致小鼠的平滑肌中δ-和ε-唾液酸和谷氨酸和α-依托莫多糖的量减少。