Inner nuclear membrane proteins: functions and targeting

被引:131
作者
Holmer, L
Worman, HJ
机构
[1] Columbia Univ Coll Phys & Surg, Dept Med, New York, NY 10032 USA
[2] Columbia Univ Coll Phys & Surg, Dept Anat & Cell Biol, New York, NY 10032 USA
关键词
nuclear envelope; nuclear lamina; Emery-Dreifuss muscular dystrophy; limb-girdle muscular dystrophy; Dunnigan-type partial lipodystrophy; intracellular protein trafficking; lamin B receptor; lamina-associated polypeptide; nurim;
D O I
10.1007/PL00000813
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
We summarize the properties of integral membrane proteins that reside in the inner nuclear membrane, including lamin B receptor (LBR), lamina-associated polypeptide (LAP) 1, LAP2, emerin, MAN1 and nurim. Most of these proteins interact with lamins and chromatin, Some data also suggest more speculative functions such as gene regulation and possibly sterol metabolism. Mutations in emerin and nuclear lamins have been associated with muscular dystrophies and lipodystrophy, raising new questions about the functions of inner nuclear membrane proteins. Integral proteins of the inner nuclear membrane are synthesized on the rough endoplasmic reticulum (ER) and reach the inner nuclear membrane by lateral diffusion in the connected ER and nuclear envelope membranes. Associations with nuclear ligands retain them in the inner nuclear membrane. Further investigation of the functions and targeting of inner nuclear membrane proteins are needed to determine how they are involved in human disease.
引用
收藏
页码:1741 / 1747
页数:7
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