Cypher knockout mice display a severe form of congenital myopathy and die postnatally from functional failure in multiple striated muscles due to failure in Z-line maintenance.
Cypher is essential for maintaining Z-line structure during muscle contraction, and its absence leads to severe congenital myopathy and postnatal death.
Cypher is a member of a recently emerging family of proteins containing a PDZ domain at their NH(2) 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 alpha-actinin or other Z-line components. These results suggest that Cypher functions as a linker-strut to maintain cytoskeletal structure during contraction.
Zhou et al. (Mon,) conducted a other in Congenital myopathy. Cypher knockout vs. Wild-type was evaluated on Phenotype of Cypher knockout mice. Cypher knockout mice display a severe form of congenital myopathy and die postnatally from functional failure in multiple striated muscles due to failure in Z-line maintenance.
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