Interaction between emerin and nuclear lamins

被引:121
作者
Sakaki, M
Koike, H
Takahashi, N
Sasagawa, N
Tomioka, S
Arahata, K
Ishiura, S
机构
[1] Univ Tokyo, Grad Sch Arts & Sci, Dept Life Sci, Meguro Ku, Tokyo 1538902, Japan
[2] Meiji Pharmaceut Univ, Dept Biochem, Tokyo 2048588, Japan
[3] Univ Tokyo, Inst Mol & Cellular Biosci, Dept Mol Biol, Bunkyo Ku, Tokyo 1130032, Japan
[4] NCNP, Natl Inst Neurosci, Dept Neuromuscular Res, Tokyo 1878502, Japan
关键词
emerin; Emery-Dreifuss muscular dystrophy; lamin; nuclear envelope protein; nuclear matrix;
D O I
10.1093/oxfordjournals.jbchem.a002860
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
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.
引用
收藏
页码:321 / 327
页数:7
相关论文
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