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Rockefeller University Press, Journal of Cell Biology, 4(155), p. 605-612, 2001

DOI: 10.1083/jcb.200107092

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

Journal article published in 2001 by Qiang Zhou, Po-Hsien Chu, Chenqun Huang, Ching-Feng Cheng ORCID, Maryann E. Martone, Gudrun Knoll, G. Diane Shelton, G. Diane Shelton, Sylvia Evans, Ju Chen
This paper is made freely available by the publisher.
This paper is made freely available by the publisher.

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Abstract

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.