Zasp/Cypher internal ZM-motif containing fragments are sufficient to co-localize with alpha-actinin--analysis of patient mutations

Exp Cell Res. 2006 May 1;312(8):1299-311. doi: 10.1016/j.yexcr.2005.12.036. Epub 2006 Feb 14.

Abstract

Z-band alternatively spliced PDZ-containing protein (ZASP/Cypher) has an important role in maintaining Z-disc stability in striated and cardiac muscle. ZASP/Cypher interacts through its PDZ domain with the major Z-disc actin cross-linker, alpha-actinin. ZASP/Cypher also has a conserved sequence called the ZM-motif, and it is found in two alternatively spliced exons 4 and 6. We have shown earlier that the ZM-motif containing internal regions of two related proteins ALP and CLP36 interact with alpha-actinin rod region, and that the ZM-motif is important in targeting ALP to the alpha-actinin containing structures in cell. Here, we show that the ZASP/Cypher internal fragments containing either ZM exon 4 or 6 co-localized with alpha-actinin in cultured myoblasts and nonmuscle cells. Fragments of 130 residues around the ZM-consensus were sufficient for localization, which is similar to our previous results of ALP. Moreover, ZASP/Cypher protein interacted directly with the alpha-actinin rod and competed with ALP in binding to the rod. During the inhibition of stress fiber assembly ZASP/Cypher and alpha-actinin co-localization could be partially disturbed, suggesting that ZASP/Cypher is bound to alpha-actinin mainly when alpha-actinin is localizing in stress fibers. Many point mutations found in cardiomyopathy patients are located in the internal region of ZASP/Cypher. However, we found no evidence that human patient mutations in the internal domain would affect the ZASP/Cypher co-localization with alpha-actinin, or that the mutations would destabilize the ZASP/Cypher protein.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Actinin / metabolism*
  • Adaptor Proteins, Signal Transducing / chemistry
  • Adaptor Proteins, Signal Transducing / genetics
  • Adaptor Proteins, Signal Transducing / metabolism*
  • Amino Acid Motifs / physiology
  • Animals
  • CHO Cells
  • Cardiomyopathies / genetics*
  • Cardiomyopathies / metabolism
  • Cardiomyopathies / physiopathology
  • Cricetinae
  • Exons / physiology
  • Humans
  • Intracellular Membranes / metabolism
  • Intracellular Membranes / ultrastructure
  • LIM Domain Proteins
  • Mice
  • Muscle, Skeletal / metabolism*
  • Muscle, Skeletal / ultrastructure
  • Mutation / genetics*
  • Myoblasts
  • Myocardium / metabolism*
  • Myocardium / ultrastructure
  • Organelles / metabolism
  • Organelles / ultrastructure
  • Peptide Fragments / chemistry
  • Peptide Fragments / metabolism
  • Protein Binding / physiology
  • Sarcomeres / metabolism
  • Sarcomeres / ultrastructure
  • Stress Fibers / metabolism

Substances

  • Adaptor Proteins, Signal Transducing
  • LDB3 protein, human
  • LIM Domain Proteins
  • Peptide Fragments
  • Actinin