The Drosophila mitochondrial translation elongation factor G1 contains a nuclear localization signal and inhibits growth and DPP signaling.
The Drosophila mitochondrial translation elongation factor G1 contains a nuclear localization signal and inhibits growth and DPP signaling.
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DOI：
10.1371/journal.pone.0016799
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发表时间：
2011-02-25
期刊：
影响因子：
3.7
通讯作者：
Haerry TE
中科院分区：
文献类型：
--
作者：
Trivigno C;Haerry TE
人线粒体延伸因子G1（EF-G1）的突变是隐性致死的，并导致出生后不久死亡。我们已经分离出的突变iconoclast（ico），编码高度保守的果蝇直系同源EF-G1。我们发现EF-G1在果蝇发育过程中是必不可少的，但它的功能并不是每个组织都需要的。与无效突变相反，错义突变表现出更强的、可能是新形态的表型，导致胚胎发生期间的过早死亡。我们的实验表明，EF-G1含有二级C-末端核定位信号。EF-G1的错义突变体形式的表达可以在细胞核中积累并导致生长和图案缺陷以及动物致死。我们发现，编码突变型人EF-G1蛋白的转基因可以拯救ICO突变体，这表明人类疾病的根本问题不仅仅是酶活性的丧失。我们的研究结果与一个模型是一致的，其中EF-G1作为一个逆行信号从线粒体到细胞核，以减缓细胞增殖，如果线粒体能量输出低。
Mutations in the human mitochondrial elongation factor G1 (EF-G1) are recessive lethal and cause death shortly after birth. We have isolated mutations in iconoclast (ico), which encodes the highly conserved Drosophila orthologue of EF-G1. We find that EF-G1 is essential during fly development, but its function is not required in every tissue. In contrast to null mutations, missense mutations exhibit stronger, possibly neomorphic phenotypes that lead to premature death during embryogenesis. Our experiments show that EF-G1 contains a secondary C-terminal nuclear localization signal. Expression of missense mutant forms of EF-G1 can accumulate in the nucleus and cause growth and patterning defects and animal lethality. We find that transgenes that encode mutant human EF-G1 proteins can rescue ico mutants, indicating that the underlying problem of the human disease is not just the loss of enzymatic activity. Our results are consistent with a model where EF-G1 acts as a retrograde signal from mitochondria to the nucleus to slow down cell proliferation if mitochondrial energy output is low.
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影响因子：
16
作者：
Tsuboi, Masafumi;Morita, Hiroyuki;Takeuchi, Nono
通讯作者：
Takeuchi, Nono
影响因子：
11.4
作者：
CZWORKOWSKI, J;WANG, J;MOORE, PB
通讯作者：
MOORE, PB
DOI：
10.1007/bf00848156
发表时间：
1984-01-01
期刊：
WILHELM ROUXS ARCHIVES OF DEVELOPMENTAL BIOLOGY
影响因子：
--
作者：
NUSSLEINVOLHARD, C;WIESCHAUS, E;KLUDING, H
通讯作者：
KLUDING, H
影响因子：
64.5
作者：
Chacinska A;Koehler CM;Milenkovic D;Lithgow T;Pfanner N
通讯作者：
Pfanner N
影响因子：
16.2
作者：
McCabe, BD;Marqués, G;O'Connor, MB
通讯作者：
O'Connor, MB