Gene Symbol: Myoz2
Description: myozenin 2
Alias: 1110012I24Rik, Fatz-2, Myozl2, myozenin-2, FATZ-related protein 2, calsarcin-1, myoz2, myozenin-like 2
Species: mouse
Products:     Myoz2

Top Publications

  1. Frey N, Richardson J, Olson E. Calsarcins, a novel family of sarcomeric calcineurin-binding proteins. Proc Natl Acad Sci U S A. 2000;97:14632-7 pubmed
    ..Calsarcins represent a novel family of sarcomeric proteins that link calcineurin with the contractile apparatus, thereby potentially coupling muscle activity to calcineurin activation. ..
  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
    ..Calsarcins represent a novel family of sarcomeric proteins that serve as focal points for the interactions of an array of proteins involved in Z-disc structure and signal transduction in striated muscle. ..
  3. Frey N, Barrientos T, Shelton J, Frank D, Rütten H, Gehring D, et al. Mice lacking calsarcin-1 are sensitized to calcineurin signaling and show accelerated cardiomyopathy in response to pathological biomechanical stress. Nat Med. 2004;10:1336-43 pubmed
    ..These findings show important roles for calsarcins as modulators of calcineurin signaling and the transmission of a specific subset of stress signals leading to cardiac remodeling in vivo. ..
  4. Faulkner G, Pallavicini A, Comelli A, Salamon M, Bortoletto G, Ievolella C, et al. FATZ, a filamin-, actinin-, and telethonin-binding protein of the Z-disc of skeletal muscle. J Biol Chem. 2000;275:41234-42 pubmed
    ..Therefore, FATZ may be the first member of a small family of novel muscle proteins. ..
  5. Frank D, Kuhn C, van Eickels M, Gehring D, Hanselmann C, Lippl S, et al. Calsarcin-1 protects against angiotensin-II induced cardiac hypertrophy. Circulation. 2007;116:2587-96 pubmed
    ..Thus, overexpression of CS1 might represent a novel approach to attenuate pathological cardiac hypertrophy. ..
  6. Dierck F, Kuhn C, Rohr C, Hille S, Braune J, Sossalla S, et al. The novel cardiac z-disc protein CEFIP regulates cardiomyocyte hypertrophy by modulating calcineurin signaling. J Biol Chem. 2017;292:15180-15191 pubmed publisher
    ..In summary, we report on a previously uncharacterized protein CEFIP that modulates calcineurin/NFAT signaling in cardiomyocytes, a finding with possible implications for the pathogenesis of cardiomyopathy. ..
  7. Ivandic B, Mastitsky S, Schönsiegel F, Bekeredjian R, Eils R, Frey N, et al. Whole-genome analysis of gene expression associates the ubiquitin-proteasome system with the cardiomyopathy phenotype in disease-sensitized congenic mouse strains. Cardiovasc Res. 2012;94:87-95 pubmed publisher
    ..Mutant alleles of the genes calsarcin-1 (Myoz2), sarcoglycan-delta (Sgcd), and muscle LIM protein (Csrp2) were each transferred onto inbred strain backgrounds ..
  8. Posch M, Thiemann L, Tomasov P, Veselka J, Cardim N, Garcia Castro M, et al. Sequence analysis of myozenin 2 in 438 European patients with familial hypertrophic cardiomyopathy. Med Sci Monit. 2008;14:CR372-4 pubmed
    ..b>MYOZ2 encodes for sarcomeric calsarcin-1 located in the myocardial z-disc, a focal point of HCM disease genes...
  9. Schoensiegel F, Bekeredjian R, Schrewe A, Weichenhan D, Frey N, Katus H, et al. Atrial natriuretic peptide and osteopontin are useful markers of cardiac disorders in mice. Comp Med. 2007;57:546-53 pubmed
    ..129S1/Sv; (2) a surgical model of nonischemic myocardial infarction; and (3) delta-sarcoglycan (Sgcd) and calsarcin 1 [also known as myozenin 2 (Myoz2)] knockout models of cardiomyopathy...

More Information


  1. Potthoff M, Arnold M, McAnally J, Richardson J, Bassel Duby R, Olson E. Regulation of skeletal muscle sarcomere integrity and postnatal muscle function by Mef2c. Mol Cell Biol. 2007;27:8143-51 pubmed
    ..These results reveal a key role for Mef2c in maintenance of sarcomere integrity and postnatal maturation of skeletal muscle. ..
  2. Paulsson A, Franklin S, Mitchell Jordan S, Ren S, Wang Y, Vondriska T. Post-translational regulation of calsarcin-1 during pressure overload-induced cardiac hypertrophy. J Mol Cell Cardiol. 2010;48:1206-14 pubmed publisher
    ..Overall, the findings expand the roles of calsarcins to include nuclear tasks during cardiac growth. ..
  3. Pelikan R, Iwata J, Suzuki A, Chai Y, Hacia J. Identification of candidate downstream targets of TGF? signaling during palate development by genome-wide transcript profiling. J Cell Biochem. 2013;114:796-807 pubmed publisher
  4. Wang H, Yang S, Yang E, Zhu Z, Mu Y, Feng S, et al. NF-kappaB mediates the transcription of mouse calsarcin-1 gene, but not calsarcin-2, in C2C12 cells. BMC Mol Biol. 2007;8:19 pubmed
    ..The calsarcins may also serve as a valuable mechanistic tool to better understand the regulation of calcineurin signalling during muscle differentiation. ..
  5. Ruggiero A, Chen S, Lombardi R, Rodriguez G, Marian A. Pathogenesis of hypertrophic cardiomyopathy caused by myozenin 2 mutations is independent of calcineurin activity. Cardiovasc Res. 2013;97:44-54 pubmed publisher
    ..We determined potential involvement of calcineurin in the pathogenesis of HCM caused by mutations in myozenin 2 (MYOZ2), an inhibitor of calcineurin...
  6. 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. ..
  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
    ..This is the first report of a binding motif common to both the myotilin and the FATZ (calsarcin/myozenin) families that is specific for interactions with Enigma family members. ..
  8. Heineke J, Ruetten H, Willenbockel C, Gross S, Naguib M, Schaefer A, et al. Attenuation of cardiac remodeling after myocardial infarction by muscle LIM protein-calcineurin signaling at the sarcomeric Z-disc. Proc Natl Acad Sci U S A. 2005;102:1655-60 pubmed
    ..Our study reveals a link between the stress sensor MLP and the calcineurin-NFAT pathway at the sarcomeric Z-disk in cardiomyocytes and indicates that reduced MLP-calcineurin signaling predisposes to adverse remodeling after MI. ..