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Published online 5 November 2001. doi:10.1083/jcb.200107092
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© The Rockefeller University Press, 0021-9525/2001/11/605 $5.00
The Journal of Cell Biology, Volume 155, Number 4, November 12, 2001 605-612

Article

 Ablation of Cypher, a PDZ-LIM domain Z-line protein, causes a severe form of congenital myopathy

Qiang Zhou1, Po-Hsien Chu1, Chenqun Huang1, Ching-Feng Cheng1, Maryann E. Martone2, Gudrun Knoll1, G. Diane Shelton3, Sylvia Evans1 and Ju Chen1

1 Institute of Molecular Medicine and Department of Medicine, University of California at San Diego School of Medicine, La Jolla, CA 92093
2 Department of Neuroscience, University of California at San Diego School of Medicine, La Jolla, CA 92093
3 Department of Pathology, University of California at San Diego School of Medicine, La Jolla, CA 92093

Address correspondence to Ju Chen, Department of Medicine, University of California at San Diego School of Medicine, 9500 Gilman Dr., La Jolla, CA 92093-0613. Tel.: (858) 822-2452. Fax: (858) 534-2069. E-mail: juchen{at}ucsd.edu

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 {alpha}-actinin or other Z-line components. These results suggest that Cypher functions as a linker-strut to maintain cytoskeletal structure during contraction.

Key Words: congenital myopathy; cypher; LIM; PDZ; Z-line


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