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- Title
Nesprin-1: novel regulator of striated muscle nuclear positioning and mechanotransduction.
- Authors
De Silva, Shanelle; Zhijuan Fan; Baoqiang Kang; Shanahan, Catherine M.; Qiuping Zhang
- Abstract
Nesprins (nuclear envelope spectrin repeat proteins) are multi-isomeric scaffolding proteins. Giant nesprin-1 and -2 localise to the outer nuclear membrane, interact with SUN (Sad1p/ UNC-84) domain-containing proteins at the inner nuclear membrane to form the LInker of Nucleoskeleton and Cytoskeleton (LINC) complex, which, in association with lamin A/C and emerin, mechanically couples the nucleus to the cytoskeleton. Despite ubiquitous expression of nesprin giant isoforms, pathogenic mutations in nesprin-1 and -2 are associated with tissue-specific disorders, particularly related to striated muscle such as dilated cardiomyopathy and Emery–Dreifuss muscular dystrophy. Recent evidence suggests this muscle-specificity might be attributable in part, to the small muscle specific isoform, nesprin-1α2, which has a novel role in striated muscle function. Our current understanding of muscle-specific functions of nesprin-1 and its isoforms will be summarised in this review to provide insight into potential pathological mechanisms of nesprin-related muscle disease and may inform potential targets of therapeutic modulation.
- Subjects
STRIATED muscle; NUCLEAR membranes; SCAFFOLD proteins; MUSCULAR dystrophy; NUCLEAR matrix; FACIOSCAPULOHUMERAL muscular dystrophy
- Publication
Biochemical Society Transactions, 2023, Vol 51, Issue 3, p1331
- ISSN
0300-5127
- Publication type
Article
- DOI
10.1042/BST20221541