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Gene Review

Dsp  -  desmoplakin

Mus musculus

Synonyms: 2300002E22Rik, 5730453H04Rik, AA407887, AA407888, AW109828, ...
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Disease relevance of Dsp


High impact information on Dsp

  • These differences were not dependent upon intercellular adhesion and were in marked contrast to keratinocytes conditionally null for another essential intercellular adhesion protein, desmoplakin (DP) [3].
  • We further show that cardiac-restricted deletion of Dsp, encoding DP, impairs cardiac morphogenesis and leads to high embryonic lethality in the homozygous state [1].
  • We established atrial myocyte cell lines expressing siRNA against desmoplakin (DP), responsible for human ARVC [1].
  • The number of epidermal desmosomes in DP-null skin is similar to wild type (WT), but they lack keratin filaments, which compromise their function [4].
  • In vitro, DP transgene expression rescues these defects [4].

Biological context of Dsp

  • We have now rescued the extra-embryonic tissues by aggregation of tetraploid (wild-type) and diploid (Dsp mutant) morulae [5].
  • In addition, the murine gene (Dsp) was mapped to mouse chromosome 13 by interspecific backcross mapping [6].
  • We present evidence that Dsg2, which is located in desmoplakin-negative wild-type ES cells in non-desmosomal junctions, is needed for normal ES cell proliferation [7].
  • This finding not only unveiled a new function for desmoplakin, but also provided the first opportunity to explore desmosome function during embryogenesis [8].
  • This study therefore defines a new family of transcription factors, the DP proteins, members of which can interact combinatorially with E2F proteins to generate an array of DNA binding complexes that integrate cell-cycle progression with the transcription apparatus through the E2F binding site [9].

Anatomical context of Dsp


Associations of Dsp with chemical compounds

  • A Triton treatment of the monolayer reveals that most of the desmoplakin present in either wild-type, keratin 8- or keratin 18-null hepatocytes is insoluble [12].
  • Together, the data indicate that a proper alignment/deposition of desmoplakin with keratins and desmoglein in hepatocytes requires keratin 8, through a reciprocal phosphoserine-dependent process [12].
  • We have shown that expression of the desmosomal protein desmoplakin decreases in the luminal uterine epithelium during the preimplantation period of pregnancy in mice [13].

Other interactions of Dsp


Analytical, diagnostic and therapeutic context of Dsp


  1. Suppression of canonical Wnt/beta-catenin signaling by nuclear plakoglobin recapitulates phenotype of arrhythmogenic right ventricular cardiomyopathy. Garcia-Gras, E., Lombardi, R., Giocondo, M.J., Willerson, J.T., Schneider, M.D., Khoury, D.S., Marian, A.J. J. Clin. Invest. (2006) [Pubmed]
  2. IgG binds to desmoglein 3 in desmosomes and causes a desmosomal split without keratin retraction in a pemphigus mouse model. Shimizu, A., Ishiko, A., Ota, T., Tsunoda, K., Amagai, M., Nishikawa, T. J. Invest. Dermatol. (2004) [Pubmed]
  3. Hyperproliferation and defects in epithelial polarity upon conditional ablation of alpha-catenin in skin. Vasioukhin, V., Bauer, C., Degenstein, L., Wise, B., Fuchs, E. Cell (2001) [Pubmed]
  4. Desmoplakin is essential in epidermal sheet formation. Vasioukhin, V., Bowers, E., Bauer, C., Degenstein, L., Fuchs, E. Nat. Cell Biol. (2001) [Pubmed]
  5. Rescuing desmoplakin function in extra-embryonic ectoderm reveals the importance of this protein in embryonic heart, neuroepithelium, skin and vasculature. Gallicano, G.I., Bauer, C., Fuchs, E. Development (2001) [Pubmed]
  6. Analysis of the desmoplakin gene reveals striking conservation with other members of the plakin family of cytolinkers. Green, K.J., Guy, S.G., Cserhalmi-Friedman, P.B., McLean, W.H., Christiano, A.M., Wagner, R.M. Exp. Dermatol. (1999) [Pubmed]
  7. Loss of desmoglein 2 suggests essential functions for early embryonic development and proliferation of embryonal stem cells. Eshkind, L., Tian, Q., Schmidt, A., Franke, W.W., Windoffer, R., Leube, R.E. Eur. J. Cell Biol. (2002) [Pubmed]
  8. Desmoplakin is required early in development for assembly of desmosomes and cytoskeletal linkage. Gallicano, G.I., Kouklis, P., Bauer, C., Yin, M., Vasioukhin, V., Degenstein, L., Fuchs, E. J. Cell Biol. (1998) [Pubmed]
  9. Molecular characterization of Xenopus laevis DP proteins. Girling, R., Bandara, L.R., Ormondroyd, E., Lam, E.W., Kotecha, S., Mohun, T., La Thangue, N.B. Mol. Biol. Cell (1994) [Pubmed]
  10. Dynamics and unexpected localization of the plakin binding protein, kazrin, in mouse eggs and early embryos. Gallicano, G.I., Foshay, K., Pengetnze, Y., Zhou, X. Dev. Dyn. (2005) [Pubmed]
  11. Targeted mutation of plakoglobin in mice reveals essential functions of desmosomes in the embryonic heart. Ruiz, P., Brinkmann, V., Ledermann, B., Behrend, M., Grund, C., Thalhammer, C., Vogel, F., Birchmeier, C., Günthert, U., Franke, W.W., Birchmeier, W. J. Cell Biol. (1996) [Pubmed]
  12. Keratin 8 modulation of desmoplakin deposition at desmosomes in hepatocytes. Loranger, A., Gilbert, S., Brouard, J.S., Magin, T.M., Marceau, N. Exp. Cell Res. (2006) [Pubmed]
  13. Desmosomes are reduced in the mouse uterine luminal epithelium during the preimplantation period of pregnancy: a mechanism for facilitation of implantation. Illingworth, I.M., Kiszka, I., Bagley, S., Ireland, G.W., Garrod, D.R., Kimber, S.J. Biol. Reprod. (2000) [Pubmed]
  14. Expressions of hepatobiliary organic anion transporters and bilirubin-conjugating enzyme in differentiating embryonic stem cells. Tanaka, Y., Yoshikawa, M., Kobayashi, Y., Kuroda, M., Kaito, M., Shiroi, A., Yamao, J., Fukui, H., Ishizaka, S., Adachi, Y. Biochem. Biophys. Res. Commun. (2003) [Pubmed]
  15. Age- and training-dependent development of arrhythmogenic right ventricular cardiomyopathy in heterozygous plakoglobin-deficient mice. Kirchhof, P., Fabritz, L., Zwiener, M., Witt, H., Sch??fers, M., Zellerhoff, S., Paul, M., Athai, T., Hiller, K.H., Baba, H.A., Breithardt, G., Ruiz, P., Wichter, T., Levkau, B. Circulation (2006) [Pubmed]
  16. Relationships between intermediate filaments and cell-specific functions in renal cell lines derived from transgenic mice harboring the temperature-sensitive T antigen. Cluzeaud, F., Bens, M., Wu, M.S., Li, Z., Vicart, P., Paulin, D., Vandewalle, A. J. Cell. Physiol. (1996) [Pubmed]
  17. Structure and function of desmosomal transmembrane core and plaque molecules. Kowalczyk, A.P., Stappenbeck, T.S., Parry, D.A., Palka, H.L., Virata, M.L., Bornslaeger, E.A., Nilles, L.A., Green, K.J. Biophys. Chem. (1994) [Pubmed]
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