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营养不良突变小鼠的坏死肌外肌纤维:肌神经接头的超微结构

Necrotic extrafusal muscle fibers of the dystrophic mutant mouse: the ultrastructure of the myoneural junction.

作者信息

Ontell M, Haller E

出版信息

Anat Rec. 1980 Aug;197(4):397-411. doi: 10.1002/ar.1091970404.

Abstract

At 28 days postpartum, the extensor digitorum longus muscle of the dy2J mutant mouse contains a population of myofibers which exhibit coagulation necrosis for approximately 90% of their length. Using the electron microscope, motor endplates were found on more than half of the necrotic fibers studied, occurring in mildly, moderately, and severely necrotic regions of these fibers. The ultrastructural features of the axonal terminals did not vary with the condition of the fiber segment at which the endplate occurred. No morphological criteria could be established for distinguishing between the axonal terminals of necrotic fibers and those of "healthy" fibers in the dystrophic animal. The principle morphological changes at motor endplates of necrotic fibers involved not the axonal terminal, but the muscle fiber itself. This study demonstrates that the necrotic myofibers, which are present at the onset of the first clinical symptoms of murine dystrophy, are innervated. Therefore, necrosis is not precipitated by structural denervation. Furthermore, observations of motor endplates on mildly, moderately, and severely necrotic regions of the myofibers indicate that regional changes along the necrotic fiber's length are not a function of distance from the motor endplate.

摘要

产后28天时,dy2J突变小鼠的趾长伸肌中有一群肌纤维,其约90%的长度呈现凝固性坏死。使用电子显微镜观察发现,在所研究的一半以上坏死纤维上存在运动终板,这些终板出现在这些纤维的轻度、中度和重度坏死区域。轴突终末的超微结构特征并不随终板所在纤维节段的状况而变化。在营养不良动物中,无法建立区分坏死纤维和“健康”纤维轴突终末的形态学标准。坏死纤维运动终板的主要形态学变化并非涉及轴突终末,而是肌纤维本身。本研究表明,在小鼠肌营养不良症首次临床症状出现时就已存在的坏死肌纤维是有神经支配的。因此,坏死并非由结构性去神经支配引发。此外,对肌纤维轻度、中度和重度坏死区域运动终板的观察表明,坏死纤维长度上的区域变化并非是与运动终板距离的函数。

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