生物
细胞生物学
肌萎缩侧索硬化
转录因子
胚胎
翻译(生物学)
TARDBP公司
胚胎发生
胚胎干细胞
RNA剪接
基因
遗传学
突变体
信使核糖核酸
SOD1
疾病
病理
医学
核糖核酸
作者
Lien-Szu Wu,Wei-Cheng Cheng,Shin-Chen Hou,Yu-Ting Yan,Shinn‐Jong Jiang,Che Kun James Shen
出处
期刊:Genesis
[Wiley]
日期:2009-12-15
卷期号:48 (1): 56-62
被引量:116
摘要
TDP-43 is a highly conserved and ubiquitously expressed nuclear protein. It has been implicated in the regulation of transcription, alternative splicing, translation, and neuronal plasticity. TDP-43 has also been shown to be a disease signature protein associated with several neurodegenerative diseases including amyotrophic lateral sclerosis. However, the correlation of the physiological functions of TDP-43 with these diseases remains unknown. We have used the gene targeting approach to disrupt the expression of TDP-43 in mouse. Loss of the TDP-43 expression results in peri-implantation lethality of mice between embryonic days (E) 3.5 and 6.5. Blastocysts of the homozygous Tardbp null mutants are morphologically normal, but exhibit defective outgrowth of the inner cell mass in vitro. Our data demonstrate the essential function of TDP-43 in peri-implantation stage during the embryo development, likely because of its involvement in multiple biological processes in a variety of cell types.
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