Repasky E A, Pollina C M, Menold M M, Hudecki M S
Proc Natl Acad Sci U S A. 1986 Feb;83(3):802-6. doi: 10.1073/pnas.83.3.802.
A significant increase in the concentration of spectrin has been observed in dystrophic chicken pectoralis major muscle when compared to normal fast-twitch muscle. In normal muscle, alpha-spectrin-specific immunofluorescence delineates each myofiber with a network pattern of staining at the sarcolemma with little staining within the cytoplasm. In dystrophic fibers, numerous intensely stained areas occur within the cytoplasm and staining at the sarcolemma is increased, thereby obscuring or eliminating the highly regular network arrangement of spectrin usually seen in this region. When immunofluorescence experiments are performed on microsomal vesicles isolated from normal and dystrophic tissues, only a small fraction of normal vesicles are stained, whereas most of the dystrophic vesicles are associated with spectrin. An increase in spectrin concentration is observed using immunoautoradiography of whole muscle and isolated microsomes, thus supporting the immunofluorescent observations described above. The early-age post-hatching when increases in spectrin concentration can be detected and the simplicity of the immunofluorescent technique make this observation useful as a new diagnostic parameter. This observation also shows that the distribution of spectrin and its concentration within nonerythroid cells can be modified by abnormal physiological states; this modification may contribute to subsequent symptoms, such as increased rigidity and abnormal calcium metabolism, that are observed in dystrophy.
与正常快肌相比,在患营养障碍的鸡胸大肌中已观察到血影蛋白浓度显著增加。在正常肌肉中,α - 血影蛋白特异性免疫荧光以肌膜处的网状染色模式勾勒出每条肌纤维,而细胞质内染色很少。在营养障碍的肌纤维中,细胞质内出现大量强染色区域,肌膜处的染色增加,从而模糊或消除了通常在此区域可见的血影蛋白高度规则的网络排列。当对从正常和营养障碍组织中分离的微粒体囊泡进行免疫荧光实验时,只有一小部分正常囊泡被染色,而大多数营养障碍囊泡与血影蛋白相关。使用全肌肉和分离的微粒体的免疫放射自显影观察到血影蛋白浓度增加,从而支持了上述免疫荧光观察结果。孵化后早期即可检测到血影蛋白浓度增加,且免疫荧光技术简单,这使得该观察结果作为一种新的诊断参数很有用。该观察结果还表明,非红细胞内血影蛋白的分布及其浓度可因异常生理状态而改变;这种改变可能导致在营养障碍中观察到的后续症状,如僵硬增加和钙代谢异常。