Interaction between emerin and nuclear lamins
Interaction between emerin and nuclear lamins
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DOI：
10.1093/oxfordjournals.jbchem.a002860
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发表时间：
2001-02-01
影响因子：
2.7
通讯作者：
Ishiura, S
中科院分区：
文献类型：
--
作者：
Sakaki, M;Koike, H;Ishiura, S
Emerin是一种参与x连锁隐性Emery-Dreifuss肌营养不良症（X-EDMD）的核膜蛋白。虽然该蛋白的功能尚不清楚，但我们发现，c端跨膜结构域截断的emerin（氨基酸1-225）与lamin A结合的亲和力高于lamin c。筛选emerin结合蛋白和免疫沉淀分析表明，lamin A特异性结合emerin。我们还使用酵母双杂交系统来澄清这种相互作用需要层粘连蛋白A尾部结构域的上半部分（氨基酸384-566）。层粘连蛋白A和层粘连蛋白C是层粘连蛋白A/C基因的选择性剪接产物，该基因负责常染色体显性埃莫里-德雷弗斯肌营养不良症（AD-EDMD）。这些结果表明，emerin-lamin相互作用需要lamin A和lamin C的尾部结构域。数据还表明，lamin - A特异性区域（567-664氨基酸）在lamin A和lamin C之间emerin结合能力的差异中起着一定的间接作用。这是第一次报道lamin A和lamin C在与emerin相互作用方面的差异。这些数据也表明核纤层蛋白A对核膜的完整性很重要。
Emerin is an inner nuclear membrane protein that is involved in X-linked recessive Emery-Dreifuss muscular dystrophy (X-EDMD). Although the function of this protein is still unknown, we revealed that C-terminus transmembrane domain-truncated emerin (amino acid 1-225) binds to lamin A with higher affinity than lamin C. Screening for the emerin binding protein and immunoprecipitation analysis showed that lamin A binds to emerin specifically. We also used the yeast two-hybrid system to clarify that this interaction requires the top half of the tail domain (amino acid 384-566) of lamin A. Lamin A and lamin C are alternative splicing products of the lamin A/C gene that is responsible for autosomal dominant Emery-Dreifuss muscular dystrophy (AD-EDMD). These results indicate that the emerin-lamin interaction requires the tail domains of lamin A and lamin C. The data also suggest that the lamin A-specific region (amino acids 567-664) plays some indirect role in the difference in emerin-binding capacity between lamin A and lamin C. This is the first report that refers the difference between lamin A and lamin C in the interaction with emerin. These data also suggest that lamin A is important for nuclear membrane integrity.