Autophagy in transition from hypertrophic cardiomyopathy to heart failure

Autophagy in transition from hypertrophic cardiomyopathy to heart failure
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DOI:
10.1093/jmicro/dfp048
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发表时间:
2010-04-01
期刊:
JOURNAL OF ELECTRON MICROSCOPY
影响因子:
--
通讯作者:
Chojnowska, Lidia
Chojnowska, Lidia
中科院分区:
其他
文献类型:
--
作者:
Fidzianska, Anna;Bilinska, Zofia T.;Chojnowska, Lidia

文献摘要

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对一例肥厚型心肌病向心力衰竭过渡阶段患者的心内膜心肌活检进行了研究。组织表现为心肌细胞肥大、萎缩,纤维化程度增加。此外,大量心肌细胞显示泛素阳性包涵体。超微结构分析表明,心肌细胞含有典型的自噬空泡,包括线粒体、糖原颗粒、降解残留物和髓鞘结构。最明显的超微结构表现是存在无定形斑块和管丝状包涵体。这种超微结构的异常使我们得出结论,自噬机制导致的心肌细胞退化导致心肌细胞死亡、丢失和心力衰竭。
Endomyocardial biopsy of a patient in transition stage from hypertrophic cardiomyopathy to heart failure was investigated. The tissue showed hypertrophy, atrophy of myocytes and an increased amount of fibrosis. In addition, numerous cardiomyocytes revealed ubiquitin positive inclusions. Ultrastructural analysis indicated that cardiomyocytes contained typical autophagic vacuoles including mitochondria, glycogen granules, degraded remnants and myelin structures. The most obvious ultrastructural finding was the presence of amorphous plaques and tubulofilamentous inclusions. Such ultrastructural abnormalities allow us to conclude that degeneration of myocardial cells by autophagy mechanisms leads to cardiomyocyte death, loss and heart failure.