Nesprins, But Not Sun Proteins, Switch Isoforms at the Nuclear Envelope During Muscle Development

被引:70
作者
Randles, K. Natalie [1 ]
Lam, Le Thanh [1 ]
Sewry, Caroline A. [1 ]
Puckelwartz, Megan [2 ]
Furling, Denis [3 ]
Wehnert, Manfred [4 ]
McNally, Elizabeth M. [2 ]
Morris, Glenn E. [1 ,5 ]
机构
[1] Robert Jones & Agnes Hunt Orthopaed Hosp, Wolfson Ctr Inherited Neuromuscular Dis, Oswestry SY10 7AG, Shrops, England
[2] Univ Chicago, Dept Human Genet, Chicago, IL 60637 USA
[3] Univ Paris 06, INSERM, CNRS, UMRS974,Inst Myol, Paris, France
[4] Inst Human Genet, D-17487 Greifswald, Germany
[5] Univ Keele, Inst Sci & Technol Med, Keele ST5 5BG, Staffs, England
关键词
nesprin; SYNE; MYNE; NUANCE; enaptin; emerin; lamin A/C; nuclear lamina; nuclear envelope; nuclear membrane; myogenesis; muscle development; NT-2; Golgi; nuclear positioning; monoclonal antibody; epitope mapping; phage display; DREIFUSS MUSCULAR-DYSTROPHY; MEMBRANE-PROTEIN; LAMIN-A/C; ACTIN CYTOSKELETON; EMERIN; ARCHITECTURE; SYNE-1; NESPRIN-1-ALPHA; LOCALIZATION; MUTATIONS;
D O I
10.1002/dvdy.22229
中图分类号
R602 [外科病理学、解剖学]; R32 [人体形态学];
学科分类号
100101 ;
摘要
Nesprins are a family of nuclear transmembrane proteins anchored via Sun proteins to the nuclear membrane. Analysis of nesprins during human muscle development revealed an increase in nesprin-1-giant during early myogenesis in vitro. During the transition from immature to mature muscle fibres in vivo, nesprin-2 partly replaced nesprin-1 at the nuclear envelope and short nesprin isoforms became dominant. Sun1 and Sun2 proteins remained unchanged during this fibre maturation. In emerin-negative skin fibroblasts, nesprin-2-giant was relocated from the nuclear envelope to the cytoplasm, not to the endoplasmic reticulum, while nesprin-1 remained at the nuclear envelope. In emerin-negative keratinocytes lacking nesprin-1, nesprin-2 remained at the nuclear envelope. HeLa cell nuclear envelopes lacked nesprin-1, which was the dominant form in myoblasts, while a novel 130-kD nesprin-2 isoform dominated Ntera-2 cells. The results suggest the possibility of isoform-specific and tissue-specific roles for nesprins in nuclear positioning. Developmental Dynamics 239:998-1009, 2010. (C) 2010 Wiley-Liss, Inc.
引用
收藏
页码:998 / 1009
页数:12
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