Cypher is a member of a recently emerging family of proteins containing a PDZ domain at their NH2 terminus and one or three LIM domains at their COOH terminus. Cypher knockout mice display a severe form of congenital myopathy and die postnatally from functional failure in multiple striated muscles. Examination of striated muscle from the mutants revealed that Cypher is not required for sarcomerogenesis or Z-line assembly, but rather is required for maintenance of the Z-line during muscle function. In vitro studies demonstrated that individual domains within Cypher localize independently to the Z-line via interactions with α-actinin or other Z-line components. These results suggest that Cypher functions as a linker-strut to maintain cytoskeletal structure during contraction.
Ablation of Cypher, a PDZ-LIM domain Z-line protein, causes a severe form of congenital myopathy
- Views Icon Views
- Share Icon Share
- Search Site
Qiang Zhou, Po-Hsien Chu, Chenqun Huang, Ching-Feng Cheng, Maryann E. Martone, Gudrun Knoll, G. Diane Shelton, Sylvia Evans, Ju Chen; Ablation of Cypher, a PDZ-LIM domain Z-line protein, causes a severe form of congenital myopathy . J Cell Biol 12 November 2001; 155 (4): 605–612. doi: https://doi.org/10.1083/jcb.200107092
Download citation file: