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肌细胞生成素基因靶向突变小鼠的肌肉缺陷与新生儿死亡

Muscle deficiency and neonatal death in mice with a targeted mutation in the myogenin gene.

作者信息

Hasty P, Bradley A, Morris J H, Edmondson D G, Venuti J M, Olson E N, Klein W H

机构信息

Institute of Molecular Genetics, Baylor College of Medicine, Houston, Texas 77030.

出版信息

Nature. 1993 Aug 5;364(6437):501-6. doi: 10.1038/364501a0.

Abstract

Myogenin is a muscle-specific transcription factor that can induce myogenesis in a variety of cell types in tissue culture. To test myogenin's role in vivo, mice homozygous for a targeted mutation in the myogenin gene were generated. These mice survive fetal development but die immediately after birth and show a severe reduction of all skeletal muscle. Myogenin-mutant mice differ from mice carrying mutations in genes for the related myogenic factors Myf5 and MyoD, which have no muscle defects. Myogenin is therefore essential for the development of functional skeletal muscle.

摘要

肌细胞生成素是一种肌肉特异性转录因子,可在组织培养中的多种细胞类型中诱导肌生成。为了测试肌细胞生成素在体内的作用,构建了肌细胞生成素基因靶向突变的纯合小鼠。这些小鼠能在胎儿发育阶段存活,但出生后立即死亡,并且所有骨骼肌都严重萎缩。肌细胞生成素突变小鼠与携带相关肌生成因子Myf5和MyoD基因突变的小鼠不同,后者没有肌肉缺陷。因此,肌细胞生成素对功能性骨骼肌的发育至关重要。

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