Residual Cajal bodies in coilin knockout mice fail to recruit Sm snRNPs and SMN, the spinal muscular atrophy gene product.
Residual Cajal bodies in coilin knockout mice fail to recruit Sm snRNPs and SMN, the spinal muscular atrophy gene product.
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螺旋蛋白基因敲除小鼠中的残留Cajal体无法募集SM SNRNPS和SMN（脊柱肌肉萎缩基因产物）。
DOI：
10.1083/jcb.200104083
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发表时间：
2001-07-23
期刊：
影响因子：
--
通讯作者：
Matera AG
中科院分区：
文献类型：
--
作者：
Tucker KE;Berciano MT;Jacobs EY;LePage DF;Shpargel KB;Rossire JJ;Chan EK;Lafarga M;Conlon RA;Matera AG
Cajal小体（CB）是一种参与小核核糖核蛋白（snRNP）生物合成的核亚细胞器。除了snRNP，它们还高度富集基础转录和细胞周期因子、核仁蛋白原纤维蛋白（Fb）和Nopp 140（Nopp）、运动神经元存活（SMN）蛋白复合物和CB标记蛋白p80卷曲蛋白。我们报告的一代敲除小鼠缺乏羧基末端487个氨基酸的卷曲蛋白。北方和Western印迹分析表明，我们已经成功地从敲除动物中去除了全长卷曲蛋白。一些纯合子突变动物是可行的，但它们的数量显着减少时，杂交到近交系背景。对突变动物的组织和细胞系的分析揭示了含有Fb和Nopp但不含其他典型核仁标志物的核仁灶的存在。这些所谓的“残留”CB既不凝聚Sm蛋白，也不招募SMN蛋白复合物的成员。野生型小鼠卷曲蛋白在敲除细胞中的瞬时表达导致CB的形成并恢复这些缺失的表位。我们的数据表明，全长卷曲蛋白是必不可少的适当形成和/或维护CB和招聘的snRNP和SMN复合物蛋白，这些核亚结构域需要卷曲蛋白COOH末端内的序列。
Cajal bodies (CBs) are nuclear suborganelles involved in the biogenesis of small nuclear ribonucleoproteins (snRNPs). In addition to snRNPs, they are highly enriched in basal transcription and cell cycle factors, the nucleolar proteins fibrillarin (Fb) and Nopp140 (Nopp), the survival motor neuron (SMN) protein complex, and the CB marker protein, p80 coilin. We report the generation of knockout mice lacking the COOH-terminal 487 amino acids of coilin. Northern and Western blot analyses demonstrate that we have successfully removed the full-length coilin protein from the knockout animals. Some homozygous mutant animals are viable, but their numbers are reduced significantly when crossed to inbred backgrounds. Analysis of tissues and cell lines from mutant animals reveals the presence of extranucleolar foci that contain Fb and Nopp but not other typical nucleolar markers. These so-called “residual” CBs neither condense Sm proteins nor recruit members of the SMN protein complex. Transient expression of wild-type mouse coilin in knockout cells results in formation of CBs and restores these missing epitopes. Our data demonstrate that full-length coilin is essential for proper formation and/or maintenance of CBs and that recruitment of snRNP and SMN complex proteins to these nuclear subdomains requires sequences within the coilin COOH terminus.
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影响因子：
4.8
作者：
Friesen, MJ;Dreyfuss, G
通讯作者：
Dreyfuss, G
影响因子：
7.8
作者：
Almeida, F;Saffrich, R;Carmo-Fonseca, M
通讯作者：
Carmo-Fonseca, M
DOI：
10.1083/jcb.118.6.1297
发表时间：
1992-09
期刊：
The Journal of cell biology
影响因子：
--
作者：
Bell P;Dabauvalle MC;Scheer U
通讯作者：
Scheer U
影响因子：
3.3
作者：
BAUER, DW;MURPHY, C;GALL, JG
通讯作者：
GALL, JG
影响因子：
3.7
作者：
BRASCH, K;OCHS, RL
通讯作者：
OCHS, RL