Gene Symbol: Xirp2
Description: xin actin-binding repeat containing 2
Alias: 2310003D02Rik, 2310008C07Rik, A530024P18Rik, AI452089, Cmya3, Gm352, Xin2, xin actin-binding repeat-containing protein 2, Xin actin-binding repeat containing 2 short splice form, beta-xin, cardiomyopathy associated 3, cardiomyopathy-associated protein 3, mXinbeta, myogenic MEF2-activated Xin-related protein, myomaxin, xeplin, xin-beta
Species: mouse
Products:     Xirp2

Top Publications

  1. Jung Ching Lin J, Gustafson Wagner E, Sinn H, Choi S, Jaacks S, Wang D, et al. Structure, Expression, and Function of a Novel Intercalated Disc Protein, Xin. J Med Sci. 2005;25:215-222 pubmed
    ..The predicted human homologue of mXin? (named Cmya3) was mapped to chromosome 2q24.3 by DNA sequencing...
  2. Huang H, Brand O, Mathew M, Ignatiou C, Ewen E, McCalmon S, et al. Myomaxin is a novel transcriptional target of MEF2A that encodes a Xin-related alpha-actinin-interacting protein. J Biol Chem. 2006;281:39370-9 pubmed
    ..This gene, called myomaxin, was identified as a gene markedly down-regulated in MEF2A knock-out hearts...
  3. Gustafson Wagner E, Sinn H, Chen Y, Wang D, Reiter R, Lin J, et al. Loss of mXinalpha, an intercalated disk protein, results in cardiac hypertrophy and cardiomyopathy with conduction defects. Am J Physiol Heart Circ Physiol. 2007;293:H2680-92 pubmed
    ..In the mouse, there are two homologous genes, mXinalpha and mXinbeta. The human homolog of mXinalpha, Cmya1, maps to chromosomal region 3p21.2-21...
  4. Tran P, Haycraft C, Besschetnova T, Turbe Doan A, Stottmann R, Herron B, et al. THM1 negatively modulates mouse sonic hedgehog signal transduction and affects retrograde intraflagellar transport in cilia. Nat Genet. 2008;40:403-410 pubmed publisher
    ..Specifically, the aln mutation uncouples the roles of anterograde and retrograde transport in SHH signaling, suggesting that anterograde IFT is required for GLI activation and that retrograde IFT modulates this event. ..
  5. McCalmon S, Desjardins D, Ahmad S, Davidoff K, Snyder C, Sato K, et al. Modulation of angiotensin II-mediated cardiac remodeling by the MEF2A target gene Xirp2. Circ Res. 2010;106:952-60 pubmed publisher
    ..Here we demonstrate that the MEF2A target gene Xirp2 (also known as cardiomyopathy associated gene 3 [CMYA3]) is an important effector of the Ang II signaling pathway in the heart...
  6. Wang Q, Lin J, Reinking B, Feng H, Chan F, Lin C, et al. Essential roles of an intercalated disc protein, mXinbeta, in postnatal heart growth and survival. Circ Res. 2010;106:1468-78 pubmed publisher
    The Xin repeat-containing proteins mXinalpha and mXinbeta localize to the intercalated disc of mouse heart and are implicated in cardiac development and function...
  7. Wang Q, Lin J, Chan S, Lin J. The Xin repeat-containing protein, mXin?, initiates the maturation of the intercalated discs during postnatal heart development. Dev Biol. 2013;374:264-80 pubmed publisher
    ..human cardiomyopathy-associated (CMYA)/Xin actin-binding repeat-containing protein (XIRP) genes (CMYA1/XIRP1 and CMYA3/XIRP2, respectively) encode proteins localized to ICDs...
  8. Duka A, Schwartz F, Duka I, Johns C, Melista E, Gavras I, et al. A novel gene (Cmya3) induced in the heart by angiotensin II-dependent but not salt-dependent hypertension in mice. Am J Hypertens. 2006;19:275-81 pubmed
    ..Through sequencing and expression analysis we found that the unknown transcript is part of the cardiomyopathy associated 3 (Cmya3) gene, being overexpressed in Ang II-induced but not salt-induced hypertension...
  9. Francis S, Krey J, Krystofiak E, Cui R, Nanda S, Xu W, et al. A short splice form of Xin-actin binding repeat containing 2 (XIRP2) lacking the Xin repeats is required for maintenance of stereocilia morphology and hearing function. J Neurosci. 2015;35:1999-2014 pubmed publisher
    ..One such protein is Xin-actin binding repeat containing 2 (XIRP2), an actin-cross-linking protein previously reported to be specifically expressed in striated muscle...

More Information


  1. Scheffer D, Zhang D, Shen J, Indzhykulian A, Karavitaki K, Xu Y, et al. XIRP2, an actin-binding protein essential for inner ear hair-cell stereocilia. Cell Rep. 2015;10:1811-8 pubmed publisher
    ..that different isoforms of this protein are expressed and differentially located: short splice forms (also called XEPLIN) are targeted more to stereocilia, whereas two long isoforms containing a XIN-repeat domain are in both ..
  2. Leber Y, Ruparelia A, Kirfel G, van der Ven P, Hoffmann B, Merkel R, et al. Filamin C is a highly dynamic protein associated with fast repair of myofibrillar microdamage. Hum Mol Genet. 2016;25:2776-2788 pubmed
    ..Analysis of cardiomyocytes from Xirp1 and Xirp2 deficient animals indicates that both Xin actin-binding repeat-containing proteins stabilize FLNc selectively in ..
  3. Huang L, Wu K, Zhang L, Wang Q, Tang S, Wu Q, et al. Critical Roles of Xirp Proteins in Cardiac Conduction and Their Rare Variants Identified in Sudden Unexplained Nocturnal Death Syndrome and Brugada Syndrome in Chinese Han Population. J Am Heart Assoc. 2018;7: pubmed publisher
    ..Human cardiomyopathy-associated 1 (XIRP1) and 3 (XIRP2) are intercalated disc-associated, Xin repeats-containing proteins...
  4. Lamberti A, Sanges C, Chambery A, Migliaccio N, Rosso F, Di Maro A, et al. Analysis of interaction partners for eukaryotic translation elongation factor 1A M-domain by functional proteomics. Biochimie. 2011;93:1738-46 pubmed publisher
    ..Interestingly, a co-localization of SORBS2 and eEF1A was evidenced at level of plasma membrane, thus suggesting the involvement of eEF1A1 in novel key signal transduction complexes. ..
  5. Grosskurth S, Bhattacharya D, Wang Q, Lin J. Emergence of Xin demarcates a key innovation in heart evolution. PLoS ONE. 2008;3:e2857 pubmed publisher
    The mouse Xin repeat-containing proteins (mXinalpha and mXinbeta) localize to the intercalated disc in the heart...