The role of inner nuclear membrane protein emerin in myogenesis

被引:0
作者
Marano, Nicholas [1 ,2 ]
Holaska, James M. [1 ,2 ]
机构
[1] Rowan Univ, Dept Biomed Sci, Cooper Med Sch, MEB 534,401 South Broadway, Camden, NJ 08103 USA
[2] Rowan Virtua Sch Translat Biomed Engn & Sci, Stratford, NJ USA
关键词
emerin; Emery-Dreifuss muscular dystrophy; laminopathy; myogenic differentiation; DREIFUSS MUSCULAR-DYSTROPHY; AUTOINTEGRATION FACTOR BAF; DEACETYLASE; 3; HDAC3; SKELETAL-MUSCLE; DOMAIN ORGANIZATION; LEM-DOMAIN; LAMIN-A; TRANSCRIPTIONAL REPRESSION; MISSENSE MUTATION; MSX1; HOMEOPROTEIN;
D O I
10.1096/fj.202500323
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
Emerin, a ubiquitously expressed inner nuclear membrane protein, plays a central role in maintaining nuclear structure and genomic organization, and in regulating gene expression and cellular signaling pathways. These functions are critical for proper myogenic differentiation and are closely linked to the pathology of Emery-Dreifuss muscular dystrophy 1 (EDMD1), a laminopathy caused by mutations in the EMD gene. Emerin, along with other nuclear lamina proteins, modulates chromatin organization, cell signaling, gene expression, and cellular mechanotransduction, processes essential for muscle development and homeostasis. Loss of emerin function disrupts chromatin localization, causes dysregulated gene expression, and alters nucleoskeletal organization, resulting in impaired myogenic differentiation. Recent findings suggest that emerin tethers repressive chromatin at the nuclear envelope, a process essential for robust myogenesis. This review provides an in-depth discussion of emerin's multifaceted roles in nuclear organization, gene regulation, and cellular signaling, highlighting its importance in myogenic differentiation and disease progression.
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页数:15
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