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首页> 外文期刊>Proceedings of the National Academy of Sciences of the United States of America >Desmin-related cardiomyopathy in transgenic mice: a cardiac amyloidosis.
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Desmin-related cardiomyopathy in transgenic mice: a cardiac amyloidosis.

机译：转基因小鼠中的结蛋白相关性心肌病：心脏淀粉样变性。

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摘要

An R120G missense mutation in the small heat shock protein alpha-B-crystallin (CryAB(R120G)) causes desmin-related cardiomyopathy (DRM). DRM is characterized by the formation of aggregates containing CryAB and desmin, and it can be recapitulated in transgenic mice by cardiac-specific expression of the mutant protein. In this article, we show that expression of CryAB(R120G) leads to the formation of electron-dense bodies characteristic of the DRMs and identify these bodies as aggresomes, which are characteristic of the neurodegenerative diseases. Cardiomyocytes transfected with adenovirus containing CryAB(R120G) establish the necessity and sufficiency of CryAB(R120G) expression for aggresome formation. The commonality of these aggresomes with oligomeric protein aggregates found in the amyloid-related degenerative diseases was corroborated by the presence of high levels of amyloid oligomers that may represent a primary toxic species in the amyloid diseases. These oligomeric amyloid intermediates are present also in cardiomyocytes derived from many human dilated and hypertrophic cardiomyopathies.
机译：小型热激蛋白α-B-晶状体蛋白（CryAB（R120G））中的R120G错义突变引起结蛋白相关性心肌病（DRM）。 DRM的特征是形成包含CryAB和结蛋白的聚集体，并且可以通过心脏特异性表达突变蛋白在转基因小鼠中重新概括。在本文中，我们表明CryAB（R120G）的表达导致形成DRMs的电子致密体，并将这些体识别为聚集体，这是神经退行性疾病的特征。用含有CryAB（R120G）的腺病毒转染的心肌细胞确定了CryAB（R120G）表达形成聚集体的必要性和充分性。这些聚集体与在淀粉样变性相关疾病中发现的寡聚蛋白聚集体的共性被高水平的淀粉样蛋白低聚物的存在所证实，这可能代表了淀粉样蛋白疾病中的主要毒性物种。这些寡聚淀粉样蛋白中间体也存在于源自许多人扩张型和肥厚型心肌病的心肌细胞中。

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