Hijikata T, Murakami T, Imamura M, Fujimaki N, Ishikawa H
Department of Anatomy, Gunma University School of Medicine, Maebashi, Gunma 371-8511, Japan.
J Cell Sci. 1999 Mar;112 ( Pt 6):867-76. doi: 10.1242/jcs.112.6.867.
Plectin is a versatile linker protein which is associated with various types of cytoskeletal components and/or filaments including intermediate filaments, and its deficiency causes the disruption of myofibrils, or muscular dystrophy. To better understand the functional role of plectin in skeletal muscle fibers, we have examined the topological and structural relationships of plectin to intermediate filaments and Z-discs in rat diaphragm muscles by confocal and immunoelectron microscopy. Immunofluorescence analysis revealed that plectin was colocalized with desmin at the periphery of Z-discs. This plectin localization around Z-discs was constantly maintained irrespective of the contracted or extended state of the muscle fibers, suggesting either direct or indirect association of plectin with Z-discs. Immunogold labeling in skinned muscle fibers clearly demonstrated that plectin-labeled fine threads linked desmin intermediate filaments to Z-discs and connected intermediate filaments to each other. These results indicate that through plectin threads desmin intermediate filaments form lateral linkages among adjacent Z-discs, preventing individual myofibrils from disruptive contraction and ensuring effective force generation.
网蛋白是一种多功能连接蛋白,它与包括中间丝在内的各种细胞骨架成分和/或细丝相关联,其缺乏会导致肌原纤维破坏或肌肉萎缩。为了更好地理解网蛋白在骨骼肌纤维中的功能作用,我们通过共聚焦显微镜和免疫电子显微镜研究了大鼠膈肌中网蛋白与中间丝和Z盘的拓扑结构关系。免疫荧光分析显示,网蛋白与结蛋白在Z盘周边共定位。无论肌纤维处于收缩状态还是伸展状态,网蛋白在Z盘周围的这种定位都能持续维持,这表明网蛋白与Z盘存在直接或间接的关联。对去表皮肌纤维进行免疫金标记清楚地表明,网蛋白标记的细丝将结蛋白中间丝连接到Z盘,并使中间丝相互连接。这些结果表明,结蛋白中间丝通过网蛋白细丝在相邻Z盘之间形成横向连接,防止单个肌原纤维发生破坏性收缩,并确保有效产生力量。