Cell Biology Division, Center of Basic Research, Biomedical Research Foundation Academy of Athens, 11527, Athens, Greece.
Histochem Cell Biol. 2013 Jul;140(1):55-63. doi: 10.1007/s00418-013-1103-z. Epub 2013 Jun 9.
Desmin, the muscle-specific intermediate filament protein, forms a 3D scaffold that links the contractile apparatus to the costameres of plasma membrane, intercalated disks, the nucleus, and also other membranous organelles. The cellular scaffold formed by desmin and its binding partners might be implicated in signaling and trafficking processes, vital mechanisms for the survival of the mammalian cell. One novel desmin-associated protein is the tripartite motif-like protein myospryn. Myospryn was initially identified as an associated partner to the biogenesis of lysosome-related organelles complex 1 protein dysbindin, implicating its potential involvement in vesicle trafficking and organelle biogenesis and/or positioning. Myospryn is also an A kinase anchoring protein, raising the possibility that together with desmin and other cytoskeletal and signaling proteins, it could participate in the subcellular targeting of protein kinase A activity in striated muscle. As with desmin, different members of this scaffold might play a crucial role in the pathogenesis of muscle disease, since any disturbance in these highly coordinated signaling pathways is expected to compromise efficient maintenance of structure-function integrity of muscle and lead to different cardiac and skeletal myopathies.
结蛋白是一种肌肉特异性中间丝蛋白，形成一个 3D 支架，将收缩装置连接到质膜的肌节、闰盘、核，以及其他膜细胞器。由结蛋白及其结合伴侣形成的细胞支架可能参与信号转导和运输过程，这是哺乳动物细胞存活的重要机制。一种新的结蛋白相关蛋白是三联基序样蛋白肌联蛋白。肌联蛋白最初被鉴定为溶酶体相关细胞器复合物 1 蛋白 dysbindin 生物发生的相关伴侣，表明其可能参与囊泡运输和细胞器生物发生和/或定位。肌联蛋白也是一种 A 激酶锚定蛋白，这增加了这样一种可能性，即它与结蛋白和其他细胞骨架和信号蛋白一起，可能参与蛋白激酶 A 在横纹肌中的亚细胞靶向活性。与结蛋白一样，这个支架的不同成员可能在肌肉疾病的发病机制中发挥关键作用，因为这些高度协调的信号通路中的任何干扰都可能破坏肌肉结构-功能完整性的有效维持，并导致不同的心脏和骨骼肌病。
