Tubular aggregates observed in spindle muscle fiber of horse lumbrical muscle
Tubular aggregates observed in spindle muscle fiber of horse lumbrical muscle
复制标题
马蚓肌梭形肌纤维中观察到的管状聚集体
作者：
W. Klomkleaw;Y. Kasashima;Ataru Kobayashi;G. Fuller;M. Morimoto;T. Nakade;M. Muto;T. Oba;R. Hamlin;M. Yamaguchi
抽象。管状聚集体（Tubular aggregate，TA）起源于肌浆网（sarcoplasmic reticulum，SR），在横截面上形成多形的双壁（或单壁）结构。TA涉及各种人类骨骼肌疾病，包括周期性麻痹、先天性肌无力综合征、炎性肌病和恶性肌增生。马蚓状肌（LM）是一种细长的梭形肌肉，由于其肢体活动有限，因此表现出不同程度的退化。在退化的纺锤体纤维中发现了双壁TA，外层TA的范围为80-116 nm（平均92 nm，n=135），内层TA的范围为50-78 nm（平均59 nm，n=135）。TA表现出肌原纤维蛋白降解、线粒体破裂伴嵴丢失、糖原积累、电子致密代谢产物、肌膜起泡外观和各种空泡的存在。LM纤维也显示出类似的退行性状态。SR结构的分解可能产生大量的SR蛋白质的积累，这些蛋白质保持为分子而不被进一步降解，并且可以聚集形成TA的有序结构。我们认为TA的形成可能是细胞在应激引起的退化中通过形成有序的聚集体来储存不平衡的额外蛋白质的一种适应。
Abstract. Tubular aggregates (TAs) originate from the sarcoplasmic reticulum (SR) and form polymorphic double (or single) -walled structures in cross section. TAs are involved in various human skeletal muscle disorders including periodic paralysis, congenital myasthenic syndromes, inflammatory myopathies, and malignant hyperthermias. Horse lumbrical muscle (LM) is a slender fusiform muscle that shows varying degrees of regression due to its limited activity in the limb. Double-walled TAs were found in degenerating spindle fibers and with a range of 80–116 nm (average 92 nm, n=135) for outer layer and 50–78 nm (average 59 nm, n=135) for the inner layer. TAs exhibit degradation of myofibrillar proteins, disruption of mitochondria with cristae lost, glycogen accumulation, electron-dense metabolic products, blebbing appearance of sarcolemma, and presence of various vacuoles. LM fibers also show a similarly degenerative state. The disassembly of the SR structure probably produces a large accumulation of SR proteins which remain as molecules without being further degraded and which could aggregate to form the orderly structure of TAs. We believe that TA formation may be an adaptation to store unbalanced extra proteins by forming ordered aggregates in degeneration caused by stress in cells.
局部肌肉退化引起的突触损失和轴突回缩。
DOI：
10.1002/(sici)1097-4695(199610)31:2
发表时间：
1996
期刊：
Journal of neurobiology
影响因子：
--
作者：
Hegstrom,CD;Truman,JW
通讯作者：
Truman,JW
烟草天蛾变态过程中类固醇对肌肉重塑的控制。
DOI：
10.1002/(sici)1097-4695(199604)29:4
发表时间：
1996
期刊：
Journal of neurobiology
影响因子：
--
作者：
Hegstrom,CD;Truman,JW
通讯作者：
Truman,JW