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

Authors: Zhou, Q  Chu, PH  Huang, C  Cheng, CF  Martone, ME  Knoll, G  Shelton, GD  Evans, S  Chen, J 
Citation: Zhou Q, etal., J Cell Biol. 2001 Nov 12;155(4):605-12. Epub 2001 Nov 5.
Pubmed: (View Article at PubMed) PMID:11696561
DOI: Full-text: DOI:10.1083/jcb.200107092

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.

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CRRD Object Information
CRRD ID: 1581815
Created: 2006-10-26
Species: All species
Last Modified: 2006-10-26
Status: ACTIVE



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