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Circulation Research ( IF 16.5 ) Pub Date : 2017-09-01 , DOI: 10.1161/res.0000000000000174
Ruth Williams

### McLendon et al identify factors influencing protein aggregation within cardiomyocytes. Accumulations of misfolded proteins within cells can impair cellular functions. Cellular aggregates of misfolded proteins are cleared mainly by the autophagic and proteasomal pathways, and this clearance is particularly important in postmitotic cells, such as those in the brain and heart. Indeed, protein aggregates are a hallmark of certain neurodegenerative disorders as well as heart conditions including desmin-related myopathy (DRM)—a disease characterized by heart and skeletal muscle degeneration. Using a mouse cardiomyocyte model of DRM and a library of silencing RNAs, McLendon and colleagues have now carried out an unbiased genome-wide screen for novel genes that either prevent protein aggregates or promote their clearance. The team identified a …

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McLendon等人确定了影响心肌细胞内蛋白质聚集的因素。细胞内错误折叠的蛋白质积累会损害细胞功能。折叠错误的蛋白质的细胞聚集体主要通过自噬和蛋白酶体途径清除,而这种清除在有丝分裂后的细胞(如大脑和心脏的细胞)中尤为重要。的确,蛋白质聚集体是某些神经退行性疾病以及心脏疾病(包括结蛋白相关性肌病(DRM))的标志,后者是一种以心脏和骨骼肌变性为特征的疾病。现在,使用DRM小鼠心肌细胞模型和沉默RNA库,McLendon及其同事现在对全基因组进行了公正的筛选,以筛选可防止蛋白质聚集或促进其清除的新基因。小组确定了……
更新日期:2017-09-04
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