Hayes James B, Bainbridge Anna M, Burnette Dylan T
Department of Cell and Developmental Biology, Vanderbilt University School of Medicine Basic Sciences, Nashville, TN, USA.
University of Tennessee, Knoxville, TN, USA.
bioRxiv. 2025 Mar 29:2025.03.28.645933. doi: 10.1101/2025.03.28.645933.
Cardiac sarcomere assembly is a highly orchestrated process requiring integration between intracellular contractile components and extracellular adhesions. While α-actinin-2 (ACTN2) is well known for its structural role at Z-discs, the function of the "non-muscle" paralog α-actinin-1 (ACTN1) in cardiomyocytes remains unclear. Using human induced pluripotent stem cell-derived cardiac myocytes (hiCMs), we demonstrate that ACTN1 is essential for sarcomere assembly. siRNA-mediated depletion of ACTN1 disrupted Z-line formation and impaired sarcomere organization, defects that were rescued by exogenous ACTN1 but not ACTN2, revealing non-redundant functions. Unlike ACTN2, ACTN1 localized predominantly to focal adhesions and was required for adhesion maturation, as evidenced by reduced adhesion size and number following ACTN1 depletion. Live-cell imaging of vinculin dynamics showed decreased stability of adhesion-associated vinculin in ACTN1-deficient cells, whereas paxillin dynamics were unaffected. These results suggest that ACTN1 stabilizes focal adhesions to promote effective force transmission during sarcomere assembly.
心肌肌节组装是一个高度协调的过程,需要细胞内收缩成分与细胞外黏附之间的整合。虽然α-辅肌动蛋白-2(ACTN2)因其在Z盘的结构作用而广为人知,但“非肌肉”旁系同源物α-辅肌动蛋白-1(ACTN1)在心肌细胞中的功能仍不清楚。利用人诱导多能干细胞衍生的心肌细胞(hiCMs),我们证明ACTN1对肌节组装至关重要。siRNA介导的ACTN1缺失破坏了Z线形成并损害了肌节组织,这些缺陷可通过外源性ACTN1而非ACTN2挽救,揭示了非冗余功能。与ACTN2不同,ACTN1主要定位于粘着斑,并且是粘着斑成熟所必需的,ACTN1缺失后粘着斑大小和数量减少证明了这一点。纽蛋白动力学的活细胞成像显示,ACTN1缺陷细胞中与粘着相关的纽蛋白稳定性降低,而桩蛋白动力学未受影响。这些结果表明,ACTN1稳定粘着斑以在肌节组装过程中促进有效的力传递。