Obscn

Summary

Gene Symbol: Obscn
Description: obscurin, cytoskeletal calmodulin and titin-interacting RhoGEF
Alias: BC046431, Gm878, UNC89, obscurin, obscn, obscurin-MLCK, obscurin-RhoGEF, obscurin-myosin light chain kinase
Species: mouse
Products:     Obscn

Top Publications

  1. Sutter S, Raeker M, Borisov A, Russell M. Orthologous relationship of obscurin and Unc-89: phylogeny of a novel family of tandem myosin light chain kinases. Dev Genes Evol. 2004;214:352-9 pubmed
    ..Recently, two novel MLCK proteins, SPEG and obscurin-MLCK, were identified with the unique feature of two tandemly-arranged MLCK domains...
  2. Hu L, Kontrogianni Konstantopoulos A. The kinase domains of obscurin interact with intercellular adhesion proteins. FASEB J. 2013;27:2001-12 pubmed publisher
    ..signaling motifs, including 2 serine/threonine kinase domains, SK1 and SK2, present at the COOH terminus of giant obscurin-B...
  3. Bowman A, Catino D, Strong J, Randall W, Kontrogianni Konstantopoulos A, Bloch R. The rho-guanine nucleotide exchange factor domain of obscurin regulates assembly of titin at the Z-disk through interactions with Ran binding protein 9. Mol Biol Cell. 2008;19:3782-92 pubmed publisher
    b>Obscurin is an approximately 800-kDa protein composed of structural and signaling domains that organizes contractile structures in striated muscle...
  4. Cunha S, Mohler P. Obscurin targets ankyrin-B and protein phosphatase 2A to the cardiac M-line. J Biol Chem. 2008;283:31968-80 pubmed publisher
    ..we identify a novel exon (exon 43') in the ankyrin-B regulatory domain that mediates interaction with the Rho-GEF obscurin. Ankyrin-B transcripts harboring exon 43' represent the primary cardiac isoform in human and mouse...
  5. Giacomello E, Sorrentino V. Localization of ank1.5 in the sarcoplasmic reticulum precedes that of SERCA and RyR: relationship with the organization of obscurin in developing sarcomeres. Histochem Cell Biol. 2009;131:371-82 pubmed publisher
    ..isoform of ankyrin1 localized on the sarcoplasmic reticulum (SR) membrane that has been shown to interact with obscurin, a sarcomeric protein. We report here studies on the localization of obscurin and ank1...
  6. Bang M, Centner T, Fornoff F, Geach A, Gotthardt M, McNabb M, et al. The complete gene sequence of titin, expression of an unusual approximately 700-kDa titin isoform, and its interaction with obscurin identify a novel Z-line to I-band linking system. Circ Res. 2001;89:1065-72 pubmed
    ..Novex-3 titin specifically interacts with obscurin, a 721-kDa myofibrillar protein composed of 57 Ig/FN3 domains, followed by one IQ, SH3, DH, and a PH domain at ..
  7. Ford Speelman D, Roche J, Bowman A, Bloch R. The rho-guanine nucleotide exchange factor domain of obscurin activates rhoA signaling in skeletal muscle. Mol Biol Cell. 2009;20:3905-17 pubmed publisher
    b>Obscurin is a large ( approximately 800-kDa), modular protein of striated muscle that concentrates around the M-bands and Z-disks of each sarcomere, where it is well positioned to sense contractile activity...
  8. Lange S, Ouyang K, Meyer G, Cui L, Cheng H, Lieber R, et al. Obscurin determines the architecture of the longitudinal sarcoplasmic reticulum. J Cell Sci. 2009;122:2640-50 pubmed publisher
    The giant protein obscurin is thought to link the sarcomere with the sarcoplasmic reticulum (SR)...
  9. Randazzo D, Giacomello E, Lorenzini S, Rossi D, Pierantozzi E, Blaauw B, et al. Obscurin is required for ankyrinB-dependent dystrophin localization and sarcolemma integrity. J Cell Biol. 2013;200:523-36 pubmed publisher
    b>Obscurin is a large myofibrillar protein that contains several interacting modules, one of which mediates binding to muscle-specific ankyrins. Interaction between obscurin and the muscle-specific ankyrin sAnk1...

More Information

Publications17

  1. Lange S, Perera S, Teh P, Chen J. Obscurin and KCTD6 regulate cullin-dependent small ankyrin-1 (sAnk1.5) protein turnover. Mol Biol Cell. 2012;23:2490-504 pubmed publisher
    ..The presence of the sAnk1.5 binding partner obscurin, and mutation of lysine residues increased sAnk1.5 protein levels, as did knockdown of KCTD6 in cardiomyocytes...
  2. Ackermann M, Hu L, Bowman A, Bloch R, Kontrogianni Konstantopoulos A. Obscurin interacts with a novel isoform of MyBP-C slow at the periphery of the sarcomeric M-band and regulates thick filament assembly. Mol Biol Cell. 2009;20:2963-78 pubmed publisher
    b>Obscurin is a multidomain protein composed of adhesion and signaling domains that plays key roles in the organization of contractile and membrane structures in striated muscles...
  3. Borisov A, Raeker M, Russell M. Developmental expression and differential cellular localization of obscurin and obscurin-associated kinase in cardiac muscle cells. J Cell Biochem. 2008;103:1621-35 pubmed
    b>Obscurin and obscurin-associated kinase are two products of the obscurin transcriptional unit that encodes a recently identified giant muscle-specific protein obscurin...
  4. Armani A, Galli S, Giacomello E, Bagnato P, Barone V, Rossi D, et al. Molecular interactions with obscurin are involved in the localization of muscle-specific small ankyrin1 isoforms to subcompartments of the sarcoplasmic reticulum. Exp Cell Res. 2006;312:3546-58 pubmed
    ..basis of the interactions between muscle-specific ankyrin1 isoforms localized on the sarcoplasmic reticulum and obscurin a protein associated with the contractile apparatus. A novel small muscle-specific ankyrin isoform, ank1...
  5. 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. ..
  6. Randazzo D, Blaauw B, Paolini C, Pierantozzi E, Spinozzi S, Lange S, et al. Exercise-induced alterations and loss of sarcomeric M-line organization in the diaphragm muscle of obscurin knockout mice. Am J Physiol Cell Physiol. 2017;312:C16-C28 pubmed publisher
    We recently reported that skeletal muscle fibers of obscurin knockout (KO) mice present altered distribution of ankyrin B (ankB), disorganization of the subsarcolemmal microtubules, and reduced localization of dystrophin at costameres...
  7. Hu L, Ackermann M, Hecker P, Prosser B, King B, O Connell K, et al. Deregulated Ca2+ cycling underlies the development of arrhythmia and heart disease due to mutant obscurin. Sci Adv. 2017;3:e1603081 pubmed publisher
    Obscurins are cytoskeletal proteins with structural and regulatory roles encoded by OBSCN. Mutations in OBSCN are associated with the development of hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM)...
  8. Ackermann M, Shriver M, Perry N, Hu L, Kontrogianni Konstantopoulos A. Obscurins: Goliaths and Davids take over non-muscle tissues. PLoS ONE. 2014;9:e88162 pubmed publisher
    ..To date, two giant obscurin isoforms have been described in detail that differ only at the extreme COOH-terminus; while obscurin-A (?720 kDa) ..