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结蛋白紊乱:猫肥厚性心肌病的一个关键特征。

Desmin disorganisation: A key feature in feline hypertrophic cardiomyopathy.

作者信息

Cheng Wan-Ching, Lawson Charlotte, Wilkie Lois, Dobromylsky Melanie, Luis Fuentes Virginia, Holt Mark R, Ehler Elisabeth, Connolly David J

机构信息

Department of Clinical Science and Services, Royal Veterinary College, Hatfield, Hertfordshire, United Kingdom.

Department of Comparative Biomedical Sciences, Royal Veterinary College, Royal College Street London, United Kingdom.

出版信息

PLoS One. 2025 Jul 14;20(7):e0327850. doi: 10.1371/journal.pone.0327850. eCollection 2025.

Abstract

Hypertrophic cardiomyopathy is usually characterised histologically by increased ventricular wall thickness and myocyte disarray. In human and rodent HCM, subcellular alterations were detected that involve the intermediate filament cytoskeleton (mainly desmin) and proteins that are important for mechanical and electrochemical connection of the cardiomyocytes (beta-catenin and connexin-43, respectively). We demonstrate here that similar changes can be visualised in HCM samples from cats, with prominent desmin and αB-crystallin aggregates that are accompanied by increased expression at the protein level. In addition, there is a disorganisation of beta-catenin and connexin-43, which display additional aberrant signals at the lateral surface of cardiomyocytes. This suggests that the subcellular response in cardiomyocytes to HCM is shared by humans and cats.

摘要

肥厚型心肌病通常在组织学上表现为心室壁厚度增加和心肌细胞排列紊乱。在人类和啮齿动物的肥厚型心肌病中,检测到亚细胞改变,涉及中间丝细胞骨架(主要是结蛋白)以及对心肌细胞机械和电化学连接很重要的蛋白质(分别为β-连环蛋白和连接蛋白43)。我们在此证明,在猫的肥厚型心肌病样本中也可以观察到类似变化,有明显的结蛋白和αB-晶状体蛋白聚集物,同时蛋白质水平的表达增加。此外,β-连环蛋白和连接蛋白43排列紊乱,在心肌细胞侧面显示出额外的异常信号。这表明人类和猫的心肌细胞对肥厚型心肌病的亚细胞反应是相同的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1d70/12258562/c09d748ea620/pone.0327850.g001.jpg

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