Specific localization of nesprin-1-α2, the short isoform of nesprin-1 with a KASH domain, in developing, fetal and regenerating muscle, using a new monoclonal antibody
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- 作者：Ian Holt ; Nguyen Thuy Duong ; Qiuping Zhang ; Le Thanh Lam…
- 关键词：SYNE1 ; Nuclear membrane ; Monoclonal antibody ; Cardiomyopathy ; Emery ; Dreifuss muscular dystrophy ; Lamin A/C
- 刊名：BMC Cell Biology
- 出版年：2016
- 出版时间：December 2016
- 年：2016
- 卷：17
- 期：1
- 全文大小：2305KB
- 刊物主题：Cell Biology; Biological Microscopy; Life Sciences, general;
- 出版者：BioMed Central
- ISSN：1471-2121
- 卷排序：17
文摘
BackgroundNesprin-1-giant (1008kD) is a protein of the outer nuclear membrane that links nuclei to the actin cytoskeleton via amino-terminal calponin homology domains. The short nesprin-1 isoform, nesprin-1-α2, is present only in skeletal and cardiac muscle and several pathogenic mutations occur within it, but the functions of this short isoform without calponin homology domains are unclear. The aim of this study was to determine mRNA levels and protein localization of nesprin-1-α2 at different stages of muscle development in order to shed light on its functions.