1JEI

LEM DOMAIN OF HUMAN INNER NUCLEAR MEMBRANE PROTEIN EMERIN

1JEI の概要

• エントリーDOI: 10.2210/pdb1jei/pdb
• 関連するPDBエントリー: 1H9E 1H9F
• NMR情報: BMRB: 5074
• 分子名称: EMERIN (1 entity in total)
• 機能のキーワード: emerin nucleus membrane domain dystrophy, membrane protein
• 細胞内の位置: Nucleus inner membrane; Single-pass membrane protein; Nucleoplasmic side: P50402
• タンパク質・核酸の鎖数: 1
• 化学式量合計: 6237.95

構造登録者

Wolff, N.,Gilquin, B.,Courchay, K.,Callebaut, I.,Zinn-Justin, S. (登録日: 2001-06-18, 公開日: 2001-07-04, 最終更新日: 2024-05-29)

主引用文献

Wolff, N.,Gilquin, B.,Courchay, K.,Callebaut, I.,Worman, H.J.,Zinn-Justin, S.
Structural analysis of emerin, an inner nuclear membrane protein mutated in X-linked Emery-Dreifuss muscular dystrophy
FEBS LETT., 501:171-176, 2001

PubMed Abstract: Like Duchenne and Becker muscular dystrophies, Emery-Dreifuss muscular dystrophy (EDMD) is characterized by myopathic and cardiomyopathic abnormalities. EDMD has the particularity of being linked to mutations in nuclear proteins. The X-linked form of EDMD is caused by mutations in the emerin gene, whereas autosomal dominant EDMD is caused by mutations in the lamin A/C gene. Emerin colocalizes with lamin A/C in interphase cells, and binds in vitro to lamin A/C. Recent work suggests that lamin A/C might serve as a receptor for emerin. We have undertaken a structural analysis of emerin, and in particular of its N-terminal domain, which is comprised in the emerin segment critical for binding to lamin A/C. We show that region 2-54 of emerin adopts the LEM fold. This fold was originally described in the two N-terminal domains of another inner nuclear membrane protein called lamina-associated protein 2 (LAP2). The existence of a conserved solvent-exposed surface on the LEM domains of LAP2 and emerin is discussed, as well as the nature of a possible common target.

PubMed: 11470279
DOI: 10.1016/S0014-5793(01)02649-7

実験手法

SOLUTION NMR