Proteins that bind A-type lamins: integrating isolated clues

被引:186
作者
Zastrow, MS [1 ]
Vlcek, S [1 ]
Wilson, KL [1 ]
机构
[1] Johns Hopkins Univ, Sch Med, Dept Cell Biol, Baltimore, MD 21205 USA
关键词
laminopathy; emerin; Emery-Dreifuss muscular dystrophy; Hutchinson-Gilford progeria syndrome; nuclear envelope;
D O I
10.1242/jcs.01102
中图分类号
Q2 [细胞生物学];
学科分类号
071009 ; 090102 ;
摘要
What do such diverse molecules as DNA, actin, retinoblastoma protein and protein kinase Calpha all have in common? They and additional partners bind 'A-type' lamins, which form stable filaments in animal cell nuclei. Mutations in A-type lamins cause a bewildering range of tissue-specific diseases, termed 'laminopathies', including Emery-Dreifuss muscular dystrophy and the devastating Hutchinson-Gilford progeria syndrome, which mimics premature aging. Considered individually and collectively, partners for A-type lamins form four loose groups: architectural partners, chromatin partners, gene-regulatory partners and signaling partners. We describe 16 partners in detail, summarize their binding sites in A-type lamins, and sketch portraits of ternary complexes and functional pathways that might depend on lamins in vivo. On the basis of our limited current knowledge, we propose lamin-associated complexes with multiple components relevant to nuclear structure (e.g. emerin, nesprin 1alpha, actin) or signaling and gene regulation (e.g. LAP2alpha, retinoblastoma, E2F-DP heterodimers, genes) as 'food for thought'. Testing these ideas will deepen our understanding of nuclear function and human disease.
引用
收藏
页码:979 / 987
页数:9
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