Gene Symbol: Ldb3
Description: LIM domain binding 3
Alias: AW742271, PDLIM6, ZASP, LIM domain-binding protein 3, PDZ-LIM domain protein, Z-band alternatively spliced PDZ-motif protein, cypher, ldb3, oracle protein, protein cypher, protein oracle
Species: mouse
Products:     Ldb3

Top Publications

  1. Faulkner G, Pallavicini A, Formentin E, Comelli A, Ievolella C, Trevisan S, et al. ZASP: a new Z-band alternatively spliced PDZ-motif protein. J Cell Biol. 1999;146:465-75 pubmed
    ..The transcript coding for this Z-band alternatively spliced PDZ motif (ZASP) protein maps on chromosome 10q22.3-10q23.2, near the locus for infantile-onset spinocerebellar ataxia.
  2. Frey N, Olson E. Calsarcin-3, a novel skeletal muscle-specific member of the calsarcin family, interacts with multiple Z-disc proteins. J Biol Chem. 2002;277:13998-4004 pubmed
    ..In addition, we show that calsarcins interact with the PDZ-LIM domain protein ZASP/Cypher/Oracle, which also localizes to the Z-disc...
  3. Huang C, Zhou Q, Liang P, Hollander M, Sheikh F, Li X, et al. Characterization and in vivo functional analysis of splice variants of cypher. J Biol Chem. 2003;278:7360-5 pubmed
    Previously, we reported two splice variants of Cypher, a striated muscle-specific PDZLIM domain protein, Cypher1 and Cypher2. We have now characterized four additional splice isoforms, two of which are novel...
  4. Passier R, Richardson J, Olson E. Oracle, a novel PDZ-LIM domain protein expressed in heart and skeletal muscle. Mech Dev. 2000;92:277-84 pubmed
    ..5. From E9.5 low expression of Oracle mRNA was detectable in myotomes. These data suggest a role for Oracle in the early development and function of heart and skeletal muscle. ..
  5. Zhou Q, Chu P, Huang C, Cheng C, Martone M, Knoll G, et al. Ablation of Cypher, a PDZ-LIM domain Z-line protein, causes a severe form of congenital myopathy. J Cell Biol. 2001;155:605-12 pubmed
    b>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...
  6. Zhou Q, Ruiz Lozano P, Martone M, Chen J. Cypher, a striated muscle-restricted PDZ and LIM domain-containing protein, binds to alpha-actinin-2 and protein kinase C. J Biol Chem. 1999;274:19807-13 pubmed
    We have cloned and characterized a novel striated muscle-restricted protein (Cypher) that has two mRNA splice variants, designated Cypher1 and Cypher2. Both proteins contain an amino-terminal PDZ domain...
  7. Lin C, Guo X, Lange S, Liu J, Ouyang K, Yin X, et al. Cypher/ZASP is a novel A-kinase anchoring protein. J Biol Chem. 2013;288:29403-13 pubmed publisher
    ..b>Cypher/ZASP, a member of PDZ-LIM domain protein family, is a cytoskeletal protein that forms multiprotein complexes at ..
  8. von Nandelstadh P, Ismail M, Gardin C, Suila H, Zara I, Belgrano A, et al. A class III PDZ binding motif in the myotilin and FATZ families binds enigma family proteins: a common link for Z-disc myopathies. Mol Cell Biol. 2009;29:822-34 pubmed publisher
    ..Mutations in Z-disc components myotilin, ZASP/Cypher, and FATZ-2 (calsarcin-1/myozenin-2) are associated with myopathies...
  9. Zheng M, Cheng H, Li X, Zhang J, Cui L, Ouyang K, et al. Cardiac-specific ablation of Cypher leads to a severe form of dilated cardiomyopathy with premature death. Hum Mol Genet. 2009;18:701-13 pubmed publisher
    ..b>Cypher/ZASP is a cytoskeletal protein localized in the sarcomeric Z-line...

Scientific Experts

More Information


  1. Bang M. Animal Models of Congenital Cardiomyopathies Associated With Mutations in Z-Line Proteins. J Cell Physiol. 2017;232:38-52 pubmed publisher
    ..Pros and cons of mouse models will be discussed and a future outlook will be given. J. Cell. Physiol. 232: 38-52, 2017. © 2016 Wiley Periodicals, Inc. ..
  2. Lin X, Miller J, Mankodi A, Kanadia R, Yuan Y, Moxley R, et al. Failure of MBNL1-dependent post-natal splicing transitions in myotonic dystrophy. Hum Mol Genet. 2006;15:2087-97 pubmed
    ..Sequestration of MBNL1, and failure to maintain these splicing transitions, has a pivotal role in the pathogenesis of muscle disease in DM. ..
  3. Cheng H, Zheng M, Peter A, Kimura K, Li X, Ouyang K, et al. Selective deletion of long but not short Cypher isoforms leads to late-onset dilated cardiomyopathy. Hum Mol Genet. 2011;20:1751-62 pubmed publisher
    b>Cypher long (CypherL) and short (CypherS) isoforms are distinguished from each other by the presence and absence of three C-terminal LIM domains, respectively...
  4. Bennett P, Maggs A, Baines A, Pinder J. The transitional junction: a new functional subcellular domain at the intercalated disc. Mol Biol Cell. 2006;17:2091-100 pubmed
    ..In particular, it provides a means for sarcomeres to be added to the ends of the cells during growth. This is of particular relevance to understanding myocyte elongation in dilated cardiomyopathy. ..
  5. Nguyen A, Xiao B, Neppl R, Kallin E, Li J, Chen T, et al. DOT1L regulates dystrophin expression and is critical for cardiac function. Genes Dev. 2011;25:263-74 pubmed publisher
    ..In addition, our study may open new avenues for the diagnosis and treatment of human heart disease. ..
  6. Seto J, Lek M, Quinlan K, Houweling P, Zheng X, Garton F, et al. Deficiency of ?-actinin-3 is associated with increased susceptibility to contraction-induced damage and skeletal muscle remodeling. Hum Mol Genet. 2011;20:2914-27 pubmed publisher
    ..In support of this, we demonstrated that the Z-disk proteins, ZASP, titin and vinculin preferentially bind to ?-actinin-2...
  7. Chamberlain C, Ranum L. Mouse model of muscleblind-like 1 overexpression: skeletal muscle effects and therapeutic promise. Hum Mol Genet. 2012;21:4645-54 pubmed publisher
    ..In summary, MBNL1 overexpression may be a valuable strategy for treating the skeletal muscle features of DM...
  8. Johnson E, Zhang L, Adams M, Phillips A, Freitas M, Froehner S, et al. Proteomic analysis reveals new cardiac-specific dystrophin-associated proteins. PLoS ONE. 2012;7:e43515 pubmed publisher
    ..In addition, our findings support the existence of cardiac-specific functions of dystrophin and may guide studies into early triggers of cardiac disease in Duchenne and Becker muscular dystrophies. ..
  9. Martinelli V, Kyle W, Kojic S, Vitulo N, Li Z, Belgrano A, et al. ZASP interacts with the mechanosensing protein Ankrd2 and p53 in the signalling network of striated muscle. PLoS ONE. 2014;9:e92259 pubmed publisher
    ..The ablation of its murine homologue Cypher results in neonatal lethality...
  10. Lin X, Ruiz J, Bajraktari I, Ohman R, Banerjee S, Gribble K, et al. Z-disc-associated, alternatively spliced, PDZ motif-containing protein (ZASP) mutations in the actin-binding domain cause disruption of skeletal muscle actin filaments in myofibrillar myopathy. J Biol Chem. 2014;289:13615-26 pubmed publisher
    ..Z-disc-associated, alternatively spliced, PDZ motif-containing protein (ZASP)/Cypher interacts with ?-actinin, myotilin, and other Z-disc proteins via the PDZ domain...
  11. Mastrototaro G, Liang X, Li X, Carullo P, Piroddi N, Tesi C, et al. Nebulette knockout mice have normal cardiac function, but show Z-line widening and up-regulation of cardiac stress markers. Cardiovasc Res. 2015;107:216-25 pubmed publisher
    ..These results suggest that the nebulette disease causing mutations have dominant gain-of-function effects. ..
  12. Mu Y, Jing R, Peter A, Lange S, Lin L, Zhang J, et al. Cypher and Enigma homolog protein are essential for cardiac development and embryonic survival. J Am Heart Assoc. 2015;4: pubmed publisher
    ..b>Cypher and its close homologue, Enigma homolog protein (ENH), are 2 Z-line proteins previously shown to be individually ..
  13. Edmondson R, Vondriska T, Biederman K, Zhang J, Jones R, Zheng Y, et al. Protein kinase C epsilon signaling complexes include metabolism- and transcription/translation-related proteins: complimentary separation techniques with LC/MS/MS. Mol Cell Proteomics. 2002;1:421-33 pubmed
    ..Most importantly, we identified two new groups of proteins that were previously unrecognized as components of the PKC epsilon complex: metabolism-related proteins and transcription/translation-related proteins. ..
  14. Li Z, Ai T, Samani K, Xi Y, Tzeng H, Xie M, et al. A ZASP missense mutation, S196L, leads to cytoskeletal and electrical abnormalities in a mouse model of cardiomyopathy. Circ Arrhythm Electrophysiol. 2010;3:646-56 pubmed publisher
    ..We previously identified a S196L mutation in exon 4 of LBD3-encoded ZASP in a family with DCM and sudden cardiac death...
  15. Scheffer D, Shen J, Corey D, Chen Z. Gene Expression by Mouse Inner Ear Hair Cells during Development. J Neurosci. 2015;35:6366-80 pubmed publisher
    ..We found that many of the known hereditary deafness genes are much more highly expressed in hair cells than surrounding cells, suggesting that genes preferentially expressed in hair cells are good candidates for unknown deafness genes. ..