Gene Symbol: Syne2
Description: spectrin repeat containing, nuclear envelope 2
Alias: 6820443O06Rik, AW546258, Cpfl8, D12Ertd777e, KASH2, NUA, Nesp2g, dice, mKIAA1011, nesprin-2, KASH domain-containing protein 2, diminished cone electroretinogram, nesprin 2, nuance, nuclear envelope spectrin repeat protein 2, nucleus and actin connecting element protein, synaptic nuclear envelope 2, synaptic nuclear envelope protein 2
Species: mouse
Products:     Syne2

Top Publications

  1. Padmakumar V, Libotte T, Lu W, Zaim H, Abraham S, Noegel A, et al. The inner nuclear membrane protein Sun1 mediates the anchorage of Nesprin-2 to the nuclear envelope. J Cell Sci. 2005;118:3419-30 pubmed
    ..Our findings propose a conserved nuclear anchorage mechanism between Caenorhabditis elegans and mammals and suggest a model in which Sun1 serves as a ;structural bridge' connecting the nuclear interior with the actin cytoskeleton. ..
  2. Zhang X, Lei K, Yuan X, Wu X, Zhuang Y, Xu T, et al. SUN1/2 and Syne/Nesprin-1/2 complexes connect centrosome to the nucleus during neurogenesis and neuronal migration in mice. Neuron. 2009;64:173-87 pubmed publisher
    ..Syne-2 mutants also display severe defects in learning and memory. These results fill an important gap in our understanding of the mechanism of nuclear movement during brain development. ..
  3. Yu J, Lei K, Zhou M, Craft C, Xu G, Xu T, et al. KASH protein Syne-2/Nesprin-2 and SUN proteins SUN1/2 mediate nuclear migration during mammalian retinal development. Hum Mol Genet. 2011;20:1061-73 pubmed publisher
    ..These key retinal developmental signaling results will advance our understanding of the mechanism of nuclear migration in the mammalian retina. ..
  4. Zhang Q, Skepper J, Yang F, Davies J, Hegyi L, Roberts R, et al. Nesprins: a novel family of spectrin-repeat-containing proteins that localize to the nuclear membrane in multiple tissues. J Cell Sci. 2001;114:4485-98 pubmed
    ..These data and structural analogies with other proteins suggest that nesprins may function as 'dystrophins of the nucleus' to maintain nuclear organization and structural integrity. ..
  5. Borrego Pinto J, Jegou T, Osorio D, Aurade F, Gorjanacz M, Koch B, et al. Samp1 is a component of TAN lines and is required for nuclear movement. J Cell Sci. 2012;125:1099-105 pubmed publisher
    ..These results support a role for Samp1 in the association between the LINC complex and lamins during nuclear movement. ..
  6. Zhang X, Xu R, Zhu B, Yang X, Ding X, Duan S, et al. Syne-1 and Syne-2 play crucial roles in myonuclear anchorage and motor neuron innervation. Development. 2007;134:901-8 pubmed
    ..mice with single and double-disruption of the KASH-domain-containing genes Syne1 (also known as Syne-1) and Syne2 (also known as Syne-2)...
  7. Luxton G, Gomes E, Folker E, Vintinner E, Gundersen G. Linear arrays of nuclear envelope proteins harness retrograde actin flow for nuclear movement. Science. 2010;329:956-9 pubmed publisher
  8. Neumann S, Schneider M, Daugherty R, Gottardi C, Eming S, Beijer A, et al. Nesprin-2 interacts with {alpha}-catenin and regulates Wnt signaling at the nuclear envelope. J Biol Chem. 2010;285:34932-8 pubmed publisher
    ..Taken together, these findings suggest novel nesprin-2 functions in cellular signaling. Moreover, we propose that, in contrast to emerin, nesprin-2 is a positive regulator of the Wnt signaling pathway. ..
  9. Apel E, Lewis R, Grady R, Sanes J. Syne-1, a dystrophin- and Klarsicht-related protein associated with synaptic nuclei at the neuromuscular junction. J Biol Chem. 2000;275:31986-95 pubmed
    ..Finally, we identify a homologous gene, syne-2, that is expressed in an overlapping but distinct pattern. ..

More Information


  1. Lüke Y, Zaim H, Karakesisoglou I, Jaeger V, Sellin L, Lu W, et al. Nesprin-2 Giant (NUANCE) maintains nuclear envelope architecture and composition in skin. J Cell Sci. 2008;121:1887-98 pubmed publisher
    ..Here, we investigate the function of Nesprin-2 Giant (NUANCE) in skin by generating mice lacking the actin-binding domain of Nesprin-2 (Nesprin-2DeltaABD)...
  2. Zhang Q, Ragnauth C, Skepper J, Worth N, Warren D, Roberts R, et al. Nesprin-2 is a multi-isomeric protein that binds lamin and emerin at the nuclear envelope and forms a subcellular network in skeletal muscle. J Cell Sci. 2005;118:673-87 pubmed
    ..Moreover, its association at the NE with lamin and emerin, the genes mutated in Emery-Dreifuss muscular dystrophy, suggests a mechanism to explain how disruption of the NE leads to muscle dysfunction. ..
  3. Haque F, Lloyd D, Smallwood D, Dent C, Shanahan C, Fry A, et al. SUN1 interacts with nuclear lamin A and cytoplasmic nesprins to provide a physical connection between the nuclear lamina and the cytoskeleton. Mol Cell Biol. 2006;26:3738-51 pubmed
    ..Our data provide evidence of a physical nuclear-cytoskeletal connection that is likely to be a key mechanism in nuclear-cytoplasmic communication and regulation of nuclear position. ..
  4. Crisp M, Liu Q, Roux K, Rattner J, Shanahan C, Burke B, et al. Coupling of the nucleus and cytoplasm: role of the LINC complex. J Cell Biol. 2006;172:41-53 pubmed
    ..with the endoplasmic reticulum, it is nevertheless enriched in several integral membrane proteins, including nesprin 2 Giant (nesp2G), an 800-kD protein featuring an NH(2)-terminal actin-binding domain...
  5. Stewart Hutchinson P, Hale C, Wirtz D, Hodzic D. Structural requirements for the assembly of LINC complexes and their function in cellular mechanical stiffness. Exp Cell Res. 2008;314:1892-905 pubmed publisher
  6. Zhen Y, Libotte T, Munck M, Noegel A, Korenbaum E. NUANCE, a giant protein connecting the nucleus and actin cytoskeleton. J Cell Sci. 2002;115:3207-22 pubmed
    b>NUANCE (NUcleus and ActiN Connecting Element) was identified as a novel protein with an alpha-actinin-like actin-binding domain. A human 21.8 kb cDNA of NUANCE spreads over 373 kb on chromosome 14q22.1-q22.3...
  7. Ostlund C, Folker E, Choi J, Gomes E, Gundersen G, Worman H. Dynamics and molecular interactions of linker of nucleoskeleton and cytoskeleton (LINC) complex proteins. J Cell Sci. 2009;122:4099-108 pubmed publisher
    ..Our data support a model in which A-type lamins and Sun2 anchor nesprin-2 in the outer nuclear membrane, whereas emerin, Sun1 and actin are dispensable for this anchoring. ..
  8. Nie S, Ke H, Gao F, Ren J, Wang M, Huo L, et al. Coiled-Coil Domains of SUN Proteins as Intrinsic Dynamic Regulators. Structure. 2016;24:80-91 pubmed publisher
    ..Thus, the two coiled-coil domains of SUN2 act as the intrinsic dynamic regulators for controlling the SUN domain activity. ..
  9. Rashmi R, Eckes B, Glockner G, Groth M, Neumann S, Gloy J, et al. The nuclear envelope protein Nesprin-2 has roles in cell proliferation and differentiation during wound healing. Nucleus. 2012;3:172-86 pubmed publisher
    ..Our findings suggest functions for Nesprin-2 at the nuclear envelope (NE) in gene regulation and in regulation of the actin cytoskeleton which impact on wound healing. ..
  10. Morimoto A, Shibuya H, Zhu X, Kim J, Ishiguro K, Han M, et al. A conserved KASH domain protein associates with telomeres, SUN1, and dynactin during mammalian meiosis. J Cell Biol. 2012;198:165-72 pubmed publisher
    ..Our study strongly suggests that the meiotic homologue-pairing mechanism mediated by the SUN-KASH NE bridge is highly conserved among eukaryotes. ..
  11. Razafsky D, Blecher N, Markov A, Stewart Hutchinson P, Hodzic D. LINC complexes mediate the positioning of cone photoreceptor nuclei in mouse retina. PLoS ONE. 2012;7:e47180 pubmed publisher
    ..This study provides key mechanistic aspects underlying the well-known spatial confinement of cone nuclei as well as a new mouse model to evaluate the pathological relevance of nuclear mispositioning. ..
  12. Banerjee I, Zhang J, Moore Morris T, Pfeiffer E, Buchholz K, Liu A, et al. Targeted ablation of nesprin 1 and nesprin 2 from murine myocardium results in cardiomyopathy, altered nuclear morphology and inhibition of the biomechanical gene response. PLoS Genet. 2014;10:e1004114 pubmed publisher
    ..Here, we identify for the first time a role for the outer nuclear membrane proteins, Nesprin 1 and Nesprin 2, in regulating gene expression in response to biomechanical load...
  13. D Alessandro M, Hnia K, Gache V, Koch C, Gavriilidis C, Rodriguez D, et al. Amphiphysin 2 Orchestrates Nucleus Positioning and Shape by Linking the Nuclear Envelope to the Actin and Microtubule Cytoskeleton. Dev Cell. 2015;35:186-98 pubmed publisher
    ..Amphiphysins thus play a central role in linking the nuclear envelope with the actin and microtubule cytoskeletons. We propose that BIN1 has a direct and evolutionarily conserved role in nuclear positioning, altered in myopathies. ..
  14. Wilson M, Holzbaur E. Nesprins anchor kinesin-1 motors to the nucleus to drive nuclear distribution in muscle cells. Development. 2015;142:218-28 pubmed publisher
  15. Arsenovic P, Ramachandran I, Bathula K, Zhu R, Narang J, Noll N, et al. Nesprin-2G, a Component of the Nuclear LINC Complex, Is Subject to Myosin-Dependent Tension. Biophys J. 2016;110:34-43 pubmed publisher
  16. Nery F, Zeng J, Niland B, Hewett J, Farley J, Irimia D, et al. TorsinA binds the KASH domain of nesprins and participates in linkage between nuclear envelope and cytoskeleton. J Cell Sci. 2008;121:3476-86 pubmed publisher
  17. Kutscheidt S, Zhu R, Antoku S, Luxton G, Stagljar I, Fackler O, et al. FHOD1 interaction with nesprin-2G mediates TAN line formation and nuclear movement. Nat Cell Biol. 2014;16:708-15 pubmed publisher
  18. Kim C, Perez A, Perkins G, Ellisman M, Dauer W. A molecular mechanism underlying the neural-specific defect in torsinA mutant mice. Proc Natl Acad Sci U S A. 2010;107:9861-6 pubmed publisher
  19. Han S, Lee J, Choi K, Hong S. Novel p104 protein regulates cell proliferation through PI3K inhibition and p27(Kip1) expression. BMB Rep. 2010;43:199-204 pubmed
    ..In summary, p104 interacts with p85alpha and is involved in the regulation of p27(Kip1) expression for the reduction of cellular proliferation. ..
  20. Randles K, Lam L, Sewry C, Puckelwartz M, Furling D, Wehnert M, et al. Nesprins, but not sun proteins, switch isoforms at the nuclear envelope during muscle development. Dev Dyn. 2010;239:998-1009 pubmed publisher
    ..The results suggest the possibility of isoform-specific and tissue-specific roles for nesprins in nuclear positioning. ..
  21. Dawe H, Adams M, Wheway G, Szymanska K, Logan C, Noegel A, et al. Nesprin-2 interacts with meckelin and mediates ciliogenesis via remodelling of the actin cytoskeleton. J Cell Sci. 2009;122:2716-26 pubmed publisher
    ..for centrosome migration and ciliogenesis interact with actin-binding isoforms of nesprin-2 (nuclear envelope spectrin repeat protein 2, also known as Syne-2 and NUANCE)...
  22. Davies R, Wagstaff K, McLaughlin E, Loveland K, Jans D. The BRCA1-binding protein BRAP2 can act as a cytoplasmic retention factor for nuclear and nuclear envelope-localizing testicular proteins. Biochim Biophys Acta. 2013;1833:3436-3444 pubmed publisher
    ..chromatin component HMG20A, nuclear mitotic apparatus protein NuMA1 and synaptic nuclear envelope protein SYNE2. Co-immunoprecipitation experiments indicate association of BRAP2 with HMG20A, NuMA1, and SYNE2 in testis, ..
  23. Chambliss A, Khatau S, Erdenberger N, Robinson D, Hodzic D, Longmore G, et al. The LINC-anchored actin cap connects the extracellular milieu to the nucleus for ultrafast mechanotransduction. Sci Rep. 2013;3:1087 pubmed publisher
    ..These results suggest an interconnected physical pathway for mechanotransduction, from the extracellular milieu to the nucleus...
  24. Maddox D, Collin G, Ikeda A, Pratt C, Ikeda S, Johnson B, et al. A Mutation in Syne2 Causes Early Retinal Defects in Photoreceptors, Secondary Neurons, and Müller Glia. Invest Ophthalmol Vis Sci. 2015;56:3776-87 pubmed publisher
    ..and characterize the pathological consequences of a spontaneous mutation named cone photoreceptor function loss 8 (cpfl8) in a mouse model with a significantly reduced cone electroretinography (ERG) response...
  25. Stroud M, Feng W, Zhang J, Veevers J, Fang X, Gerace L, et al. Nesprin 1α2 is essential for mouse postnatal viability and nuclear positioning in skeletal muscle. J Cell Biol. 2017;216:1915-1924 pubmed publisher
    ..Furthermore, we revealed that kinesin 1 was displaced in fibers of nesprin 1α2-knockout mice, suggesting that this interaction may play an important role in positioning of myonuclei and functional skeletal muscle. ..