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

doi:10.1083/jcb.200107092

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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 proteinscontaining a PDZ domain at their NH₂ terminus and one or threeLIM domains at their COOH terminus. Cypher knockout mice displaya severe form of congenital myopathy and die postnatally fromfunctional failure in multiple striated muscles. Examinationof striated muscle from the mutants revealed that Cypher isnot required for sarcomerogenesis or Z-line assembly, but ratheris required for maintenance of the Z-line during muscle function.In vitro studies demonstrated that individual domains withinCypher localize independently to the Z-line via interactionswith ${alpha}$ -actinin or other Z-line components. These results suggestthat Cypher functions as a linker-strut to maintain cytoskeletalstructure during contraction.

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

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