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过度收缩和肌动蛋白丝过多。肥厚型心肌病的基本要素。

Overcontraction and excess actin filaments. Basic elements of hypertrophic cardiomyopathy.

作者信息

Harmjanz D, Reale E

出版信息

Br Heart J. 1981 May;45(5):494-9. doi: 10.1136/hrt.45.5.494.

Abstract

Endomyocardial biopsies were taken from the right ventricular aspect of the interventricular septum in three patients with hypertrophic cardiomyopathy and were subjected to electronmicroscopical examination. Longitudinal sections confirmed already well-established findings. In the transverse sections disarray in the arrangement of the actin filaments and expansion of the myosin lattice, indicating clear overcontraction, were observed. The number of actin filaments varied from seven to 14 per hexagon; a number exceeding 12, however, was found in only one case. From our findings we conclude that overcontraction leads to a progressive deviation of the action filaments during systole caused by double overlap. The majority of these mechanisms results in a "self-impeding contraction" of the fibres. Functionally the excess of actin filaments may provide a balance between the unequal forces of contraction.

摘要

对三名肥厚型心肌病患者,从室间隔右心室面取心内膜心肌活检组织,并进行电子显微镜检查。纵切片证实了已明确的发现。在横切片中,观察到肌动蛋白丝排列紊乱以及肌球蛋白晶格扩张,表明明显过度收缩。每个六边形的肌动蛋白丝数量从7根到14根不等;然而,仅在一例中发现数量超过12根。根据我们的发现,我们得出结论,过度收缩导致收缩期肌动蛋白丝因双重重叠而逐渐偏离。这些机制中的大多数导致纤维的“自我阻碍收缩”。从功能上讲,多余的肌动蛋白丝可能在不等的收缩力之间提供平衡。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3916/482555/19fa2bb7041d/brheartj00183-0022-a.jpg

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