Ldb3

Summary

Gene Symbol: Ldb3
Description: LIM domain binding 3
Alias: RGD1564875, ZASP, LIM domain-binding protein 3, Cypher
Species: rat
Products:     Ldb3

Top Publications

  1. 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...
  2. 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.
  3. Levitas A, Konstantino Y, Muhammad E, Afawi Z, Marc Weinstein J, Amit G, et al. D117N in Cypher/ZASP may not be a causative mutation for dilated cardiomyopathy and ventricular arrhythmias. Eur J Hum Genet. 2016;24:666-71 pubmed publisher
    ..b>Cypher/ZASP is a cytoskeletal protein localized in the sarcomeric Z-line that has a pivotal role in maintaining adult ..
  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. Holmes W, Moncman C. Nebulette interacts with filamin C. Cell Motil Cytoskeleton. 2008;65:130-42 pubmed
    ..We have identified filamin-C, ZASP-1, and tropomyosin-1 as binding partners...
  6. Walker J. Protein scaffolds, lipid domains and substrate recognition in protein kinase C function: implications for rational drug design. Handb Exp Pharmacol. 2008;:185-203 pubmed publisher
  7. 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...
  8. Arimura T, Inagaki N, Hayashi T, Shichi D, Sato A, Hinohara K, et al. Impaired binding of ZASP/Cypher with phosphoglucomutase 1 is associated with dilated cardiomyopathy. Cardiovasc Res. 2009;83:80-8 pubmed publisher
    Z-band alternatively spliced PDZ-motif protein (ZASP)/Cypher is a Z-disc component of which several dilated cardiomyopathy (DCM)-associated mutations have been reported...
  9. 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...

More Information

Publications14

  1. Arimura T, Hayashi T, Terada H, Lee S, Zhou Q, Takahashi M, et al. A Cypher/ZASP mutation associated with dilated cardiomyopathy alters the binding affinity to protein kinase C. J Biol Chem. 2004;279:6746-52 pubmed
    ..b>Cypher/ZASP appears to be an ideal candidate for the cardiomyopathy causative gene, because Cypher/ZASP encodes a Z-disc ..
  2. 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...
  3. Poole A, Pula G, Hers I, Crosby D, Jones M. PKC-interacting proteins: from function to pharmacology. Trends Pharmacol Sci. 2004;25:528-35 pubmed
    ..In this article, we review recent work on C-KIPs and the implications for pharmacological and therapeutic development. ..
  4. Griggs R, Vihola A, Hackman P, Talvinen K, Haravuori H, Faulkner G, et al. Zaspopathy in a large classic late-onset distal myopathy family. Brain. 2007;130:1477-84 pubmed
    ..have a more distal than proximal muscle phenotype and a proportion of these may have mutations in myotilin, ZASP or filamin C, besides previously known desmin and alphaB-crystallin...
  5. Yamashita Y, Matsuura T, Kurosaki T, Amakusa Y, Kinoshita M, Ibi T, et al. LDB3 splicing abnormalities are specific to skeletal muscles of patients with myotonic dystrophy type 1 and alter its PKC binding affinity. Neurobiol Dis. 2014;69:200-5 pubmed publisher
    ..Here, we demonstrate the occurrence of aberrant splicing of LIM domain binding 3 (LDB3) exon 11 in DM1 skeletal muscle...