desmosomes

Summary

Summary: A type of junction that attaches one cell to its neighbor. One of a number of differentiated regions which occur, for example, where the cytoplasmic membranes of adjacent epithelial cells are closely apposed. It consists of a circular region of each membrane together with associated intracellular microfilaments and an intercellular material which may include, for example, mucopolysaccharides. (From Glick, Glossary of Biochemistry and Molecular Biology, 1990; Singleton & Sainsbury, Dictionary of Microbiology and Molecular Biology, 2d ed)

Top Publications

  1. Pilichou K, Nava A, Basso C, Beffagna G, Bauce B, Lorenzon A, et al. Mutations in desmoglein-2 gene are associated with arrhythmogenic right ventricular cardiomyopathy. Circulation. 2006;113:1171-9 pubmed
    ..The presence of mutations in desmosomal encoding genes in 40% of cases confirms that many forms of ARVC are due to alterations in the desmosome complex. ..
  2. Schwarz J, Ayim A, Schmidt A, Jäger S, Koch S, Baumann R, et al. Differential expression of desmosomal plakophilins in various types of carcinomas: correlation with cell type and differentiation. Hum Pathol. 2006;37:613-22 pubmed
    ..For PKP 1, a suppressor function of malignant behavior seems conceivable, whereas the putative functional significance of its occurrence in tumor cell nuclei requires further studies. ..
  3. Yu C, Yu C, Hsueh C, Yang C, Juang J, Hwang J, et al. Arrhythmogenic right ventricular dysplasia: clinical characteristics and identification of novel desmosome gene mutations. J Formos Med Assoc. 2008;107:548-58 pubmed publisher
    ..Patients with ARVD in Taiwan had similar clinical and cardiac manifestations as reported in the Western literature. More than half of the patients had desmosome gene mutations. ..
  4. Bauce B, Nava A, Beffagna G, Basso C, Lorenzon A, Smaniotto G, et al. Multiple mutations in desmosomal proteins encoding genes in arrhythmogenic right ventricular cardiomyopathy/dysplasia. Heart Rhythm. 2010;7:22-9 pubmed publisher
    ..Even if multiple-mutation carriers show a wide variability in clinical expression, the extent of the disease is higher compared to that in single-mutation carriers. ..
  5. Jennings J, Tucker D, Kottke M, Saito M, Delva E, Hanakawa Y, et al. Desmosome disassembly in response to pemphigus vulgaris IgG occurs in distinct phases and can be reversed by expression of exogenous Dsg3. J Invest Dermatol. 2011;131:706-18 pubmed publisher
    ..These data support a model in which PV IgG cause the loss of cell adhesion by altering the dynamics of Dsg3 assembly into desmosomes and the turnover of cell surface pools of Dsg3 through endocytic pathways.
  6. Petrof G, Mellerio J, McGrath J. Desmosomal genodermatoses. Br J Dermatol. 2012;166:36-45 pubmed publisher
    b>Desmosomes are intercellular junctions that contribute to cell-cell adhesion, signalling, development and differentiation in various tissues, including the skin...
  7. Hachem J, Wagberg F, Schmuth M, Crumrine D, Lissens W, Jayakumar A, et al. Serine protease activity and residual LEKTI expression determine phenotype in Netherton syndrome. J Invest Dermatol. 2006;126:1609-21 pubmed
    ..These studies provide a mechanistic basis for phenotypic variations in NS, and describe compensatory mechanisms that permit survival of NS patients in the face of unrelenting SP attack. ..
  8. Bass Zubek A, Hobbs R, Amargo E, Garcia N, Hsieh S, Chen X, et al. Plakophilin 2: a critical scaffold for PKC alpha that regulates intercellular junction assembly. J Cell Biol. 2008;181:605-13 pubmed publisher
    ..contact, PKP2 associates with DP in plaque precursors that form in the cytoplasm and translocate to nascent desmosomes. Here, we provide evidence that PKP2 governs DP assembly dynamics by scaffolding a DP-PKP2-protein kinase C ..
  9. Elliott P, O Mahony C, Syrris P, Evans A, Rivera Sorensen C, Sheppard M, et al. Prevalence of desmosomal protein gene mutations in patients with dilated cardiomyopathy. Circ Cardiovasc Genet. 2010;3:314-22 pubmed publisher
    ..This study suggests that both clinical presentations can be caused by mutations in desmosomal protein genes. ..

More Information

Publications107 found, 100 shown here

  1. Gehmlich K, Lambiase P, Asimaki A, Ciaccio E, Ehler E, Syrris P, et al. A novel desmocollin-2 mutation reveals insights into the molecular link between desmosomes and gap junctions. Heart Rhythm. 2011;8:711-8 pubmed publisher
    Cellular adhesion mediated by cardiac desmosomes is a prerequisite for proper electric propagation mediated by gap junctions in the myocardium. However, the molecular principles underlying this interdependence are not fully understood...
  2. Al Amoudi A, Castaño Diez D, Devos D, Russell R, Johnson G, Frangakis A. The three-dimensional molecular structure of the desmosomal plaque. Proc Natl Acad Sci U S A. 2011;108:6480-5 pubmed publisher
    ..We used cryoelectron tomography of vitreous sections from human epidermis to record 3D images of desmosomes in vivo and in situ at molecular resolution...
  3. Roberts B, Reddy R, Wahl J. Stratifin (14-3-3 ?) limits plakophilin-3 exchange with the desmosomal plaque. PLoS ONE. 2013;8:e77012 pubmed publisher
    b>Desmosomes are prominent cell-cell adhesive junctions in stratified squamous epithelia and disruption of desmosomal adhesion has been shown to have dramatic effects on the function and integrity of these tissues...
  4. Syrris P, Ward D, Evans A, Asimaki A, Gandjbakhch E, Sen Chowdhry S, et al. Arrhythmogenic right ventricular dysplasia/cardiomyopathy associated with mutations in the desmosomal gene desmocollin-2. Am J Hum Genet. 2006;79:978-84 pubmed
    ..For the first time, we have identified mutations in desmocollin-2 in patients with ARVD/C, a finding that is consistent with the hypothesis that ARVD/C is a disease of the desmosome. ..
  5. Xu T, Yang Z, Vatta M, Rampazzo A, Beffagna G, Pilichou K, et al. Compound and digenic heterozygosity contributes to arrhythmogenic right ventricular cardiomyopathy. J Am Coll Cardiol. 2010;55:587-97 pubmed publisher
    ..Disturbed junctional cytoarchitecture in subjects with desmosomal mutations confirms that ARVC is a disease of the desmosome and cell junction. ..
  6. Yin T, Green K. Regulation of desmosome assembly and adhesion. Semin Cell Dev Biol. 2004;15:665-77 pubmed
    b>Desmosomes are highly organized intercellular junctions that provide mechanical integrity to tissues by anchoring intermediate filaments to sites of strong adhesion...
  7. Getsios S, Amargo E, Dusek R, Ishii K, Sheu L, Godsel L, et al. Coordinated expression of desmoglein 1 and desmocollin 1 regulates intercellular adhesion. Differentiation. 2004;72:419-33 pubmed
    Desmoglein 1 (Dsg1) is a component of desmosomes present in the upper epidermis and can be targeted by autoimmune antibodies or bacterial toxins, resulting in skin blistering diseases...
  8. Dusek R, Godsel L, Green K. Discriminating roles of desmosomal cadherins: beyond desmosomal adhesion. J Dermatol Sci. 2007;45:7-21 pubmed
    ..adhesion molecules that cooperate to make up the adhesive core of intercellular junctions known as desmosomes. The roles of desmosomal cadherins in epidermal integrity and as targets in human cutaneous disease have been ..
  9. Nie Z, Merritt A, Rouhi Parkouhi M, Tabernero L, Garrod D. Membrane-impermeable cross-linking provides evidence for homophilic, isoform-specific binding of desmosomal cadherins in epithelial cells. J Biol Chem. 2011;286:2143-54 pubmed publisher
    b>Desmosomes and adherens junctions are cadherin-based protein complexes responsible for cell-cell adhesion of epithelial cells...
  10. Borrmann C, Grund C, Kuhn C, Hofmann I, Pieperhoff S, Franke W. The area composita of adhering junctions connecting heart muscle cells of vertebrates. II. Colocalizations of desmosomal and fascia adhaerens molecules in the intercalated disk. Eur J Cell Biol. 2006;85:469-85 pubmed
  11. Hall C, Li S, Li H, Creason V, Wahl J. Arrhythmogenic right ventricular cardiomyopathy plakophilin-2 mutations disrupt desmosome assembly and stability. Cell Commun Adhes. 2009;16:15-27 pubmed publisher
    ..b>Desmosomes are prominent cell-cell junctions found in a variety of tissues that resist mechanical stress, including the ..
  12. Aoyama Y, Nagai M, Kitajima Y. Binding of pemphigus vulgaris IgG to antigens in desmosome core domains excludes immune complexes rather than directly splitting desmosomes. Br J Dermatol. 2010;162:1049-55 pubmed publisher
    ..We examined whether or not PV IgG binding to Dsg3 directly impairs the adhesion of desmosomes. For immunofluorescence microscopy, keratinocytes were first incubated with PV IgG for 30 min in low Ca(2+) ..
  13. Nekrasova O, Amargo E, Smith W, Chen J, Kreitzer G, Green K. Desmosomal cadherins utilize distinct kinesins for assembly into desmosomes. J Cell Biol. 2011;195:1185-203 pubmed publisher
    ..desmogleins (Dsgs) and desmocollins (Dscs), comprise the adhesive core of intercellular junctions known as desmosomes. Although these adhesion molecules are known to be critical for tissue integrity, mechanisms that coordinate ..
  14. Papagerakis S, Shabana A, Pollock B, Papagerakis P, Depondt J, Berdal A. Altered desmoplakin expression at transcriptional and protein levels provides prognostic information in human oropharyngeal cancer. Hum Pathol. 2009;40:1320-9 pubmed publisher
    ..Characterizing DSP expression may improve evaluation risk of distant metastasis formation in oral cancer patients. ..
  15. BEAUDRY V, Jiang D, Dusek R, Park E, Knezevich S, Ridd K, et al. Loss of the p53/p63 regulated desmosomal protein Perp promotes tumorigenesis. PLoS Genet. 2010;6:e1001168 pubmed publisher
    ..the functional consequence of desmosome inactivation for tumorigenesis are essential for elucidating the role of desmosomes in cancer development...
  16. Spindler V, Waschke J. Role of Rho GTPases in desmosomal adhesion and pemphigus pathogenesis. Ann Anat. 2011;193:177-80 pubmed publisher
    b>Desmosomes are distinct intercellular contacts essential to the integrity of epithelial tissues and the heart muscle...
  17. Rickelt S, Moll I, Franke W. Intercellular adhering junctions with an asymmetric molecular composition: desmosomes connecting Merkel cells and keratinocytes. Cell Tissue Res. 2011;346:65-77 pubmed publisher
    ..As MCs are connected to the adjacent keratinocytes by a series of adhering junctions (AJs), of which the desmosomes are the most prominent, these junctions represent heterotypic cell-cell connections, i.e...
  18. Asimaki A, Tandri H, Duffy E, Winterfield J, Mackey Bojack S, Picken M, et al. Altered desmosomal proteins in granulomatous myocarditis and potential pathogenic links to arrhythmogenic right ventricular cardiomyopathy. Circ Arrhythm Electrophysiol. 2011;4:743-52 pubmed publisher
    ..To determine whether cytokines might promote dislocation of plakoglobin from desmosomes, we incubated cultures of neonatal rat ventricular myocytes with selected inflammatory mediators...
  19. Kitajima Y. New insights into desmosome regulation and pemphigus blistering as a desmosome-remodeling disease. Kaohsiung J Med Sci. 2013;29:1-13 pubmed publisher
    b>Desmosomes in keratinocytes are the most important intercellular adhering junctions that provide structural strength for the epidermis. These junctions are connected directly with desmosomal cadherin proteins...
  20. Scothern A, Garrod D. Visualization of desmosomes in the electron microscope. Methods Cell Biol. 2008;88:347-66 pubmed publisher
    b>Desmosomes are intercellular junctions found in epithelia and some other tissues. Their primary function is strong cell-cell adhesion...
  21. Klessner J, Desai B, Amargo E, Getsios S, Green K. EGFR and ADAMs cooperate to regulate shedding and endocytic trafficking of the desmosomal cadherin desmoglein 2. Mol Biol Cell. 2009;20:328-37 pubmed publisher
  22. Noorman M, van der Heyden M, van Veen T, Cox M, Hauer R, De Bakker J, et al. Cardiac cell-cell junctions in health and disease: Electrical versus mechanical coupling. J Mol Cell Cardiol. 2009;47:23-31 pubmed publisher
    ..It is shown that impaired electrical coupling does not change mechanical coupling. However, impaired mechanical coupling largely affects electrical coupling. ..
  23. Hatakeyama S, Hayashi S, Yoshida Y, Otsubo A, Yoshimoto K, Oikawa Y, et al. Retinoic acid disintegrated desmosomes and hemidesmosomes in stratified oral keratinocytes. J Oral Pathol Med. 2004;33:622-8 pubmed
    ..The effects of all-trans RA on cell-cell and cell-matrix connections of gingival epithelial (GE)1 cells in a multilayered culture were investigated...
  24. Yang Z, Bowles N, Scherer S, Taylor M, Kearney D, Ge S, et al. Desmosomal dysfunction due to mutations in desmoplakin causes arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circ Res. 2006;99:646-55 pubmed
    ..These data suggest DSP expression in cardiomyocytes is crucial for maintaining cardiac tissue integrity, and DSP abnormalities result in ARVD/C by cardiomyocyte death, changes in lipid metabolism, and defects in cardiac development. ..
  25. Moll R, Sievers E, Hämmerling B, Schmidt A, Barth M, Kuhn C, et al. Endothelial and virgultar cell formations in the mammalian lymph node sinus: endothelial differentiation morphotypes characterized by a special kind of junction (complexus adhaerens). Cell Tissue Res. 2009;335:109-41 pubmed publisher
    ..Finally, physiological and pathological alterations of the SEVC system will be presented, and the possible value of the molecular markers described in histological diagnoses of autochthonous lymph node tumors will be discussed...
  26. Sen Chowdhry S, Syrris P, McKenna W. Genetics of right ventricular cardiomyopathy. J Cardiovasc Electrophysiol. 2005;16:927-35 pubmed
    ..b>Desmosomes are protein complexes that anchor intermediate filaments to the cytoplasmic membrane in adjoining cells, thereby ..
  27. Cirillo N, Lanza M, De Rosa A, Cammarota M, La Gatta A, Gombos F, et al. The most widespread desmosomal cadherin, desmoglein 2, is a novel target of caspase 3-mediated apoptotic machinery. J Cell Biochem. 2008;103:598-606 pubmed
    ..Furthermore, cells pretreated with z-DEVD-fmk retained the membrane labeling of Dsg2. Taken together, our data demonstrate that the apoptotic processing of Dsg2 is mediated by caspase 3 in epithelial cells. ..
  28. Al Amoudi A, Díez D, Betts M, Frangakis A. The molecular architecture of cadherins in native epidermal desmosomes. Nature. 2007;450:832-7 pubmed
    b>Desmosomes are cadherin-based adhesive intercellular junctions, which are present in tissues such as heart and skin. Despite considerable efforts, the molecular interfaces that mediate adhesion remain obscure...
  29. Saffitz J. Arrhythmogenic cardiomyopathy and abnormalities of cell-to-cell coupling. Heart Rhythm. 2009;6:S62-5 pubmed publisher
  30. Oktarina D, van der Wier G, Diercks G, Jonkman M, Pas H. IgG-induced clustering of desmogleins 1 and 3 in skin of patients with pemphigus fits with the desmoglein nonassembly depletion hypothesis. Br J Dermatol. 2011;165:552-62 pubmed publisher
    ..PV, Dsg1 clustering, but not Dsg3 clustering, correlates with nonacantholytic intercellular widening between desmosomes. In the patient the Dsg becomes sequestered from desmosomal components which fits in with the desmoglein ..
  31. Yin T, Getsios S, Caldelari R, Godsel L, Kowalczyk A, Muller E, et al. Mechanisms of plakoglobin-dependent adhesion: desmosome-specific functions in assembly and regulation by epidermal growth factor receptor. J Biol Chem. 2005;280:40355-63 pubmed
    ..of the Armadillo family of adhesion/signaling proteins that can be incorporated into both adherens junctions and desmosomes. Loss of PG results in defects in the mechanical integrity of heart and skin and decreased adhesive strength in ..
  32. Thomason H, Cooper N, Ansell D, Chiu M, Merrit A, Hardman M, et al. Direct evidence that PKC? positively regulates wound re-epithelialization: correlation with changes in desmosomal adhesiveness. J Pathol. 2012;227:346-56 pubmed publisher
    ..We demonstrate mechanistic conservation in acute human wounds where PKC? localizes to wound edge desmosomes, which become Ca(2+) -dependent...
  33. Mao X, Choi E, Payne A. Disruption of desmosome assembly by monovalent human pemphigus vulgaris monoclonal antibodies. J Invest Dermatol. 2009;129:908-18 pubmed publisher
    ..Electron microscopy demonstrated a lack of well-formed desmosomes in keratinocytes treated with pathogenic compared to nonpathogenic mAbs...
  34. Fassihi H, Wong T, Wessagowit V, McGrath J, Mellerio J. Target proteins in inherited and acquired blistering skin disorders. Clin Exp Dermatol. 2006;31:252-9 pubmed
    ..The major adhesion units that achieve this are the hemidesmosomes and desmosomes, but when these structures are disrupted, e.g...
  35. Li D, Liu Y, Maruyama M, Zhu W, Chen H, Zhang W, et al. Restrictive loss of plakoglobin in cardiomyocytes leads to arrhythmogenic cardiomyopathy. Hum Mol Genet. 2011;20:4582-96 pubmed publisher
    ..Ultra-structural analyses revealed that desmosomes were absent in Jup mutant myocardia, whereas adherens junctions and gap junctions were preserved...
  36. Zhang M, Tavora F, Oliveira J, Li L, Franco M, Fowler D, et al. PKP2 mutations in sudden death from arrhythmogenic right ventricular cardiomyopathy (ARVC) and sudden unexpected death with negative autopsy (SUDNA). Circ J. 2012;76:189-94 pubmed
    ..E540, p.S615T). PKP2 mutations are not specific for ARVC and may result in SUDNA. The link between ARVC and desmosomal mutations may not be causal but related to an association between defective desmosomal proteins and arrhythmias. ..
  37. Schlüter H, Wepf R, Moll I, Franke W. Sealing the live part of the skin: the integrated meshwork of desmosomes, tight junctions and curvilinear ridge structures in the cells of the uppermost granular layer of the human epidermis. Eur J Cell Biol. 2004;83:655-65 pubmed
    ..of the cells of the stratum granulosum are connected by an extended subapical complex system integrating desmosomes and TJ structures identified as sites of close membrane-membrane contact and as regions of membrane-to-membrane ..
  38. Meurs K, Ederer M, Stern J. Desmosomal gene evaluation in Boxers with arrhythmogenic right ventricular cardiomyopathy. Am J Vet Res. 2007;68:1338-41 pubmed
    ..To sequence the exonic and splice site regions of the 4 desmosomal genes associated with the human form of familial arrhythmogenic right ventricular cardiomyopathy (ARVC) in Boxers with ARVC and identify a causative mutation...
  39. McCrea P, Gu D, Balda M. Junctional music that the nucleus hears: cell-cell contact signaling and the modulation of gene activity. Cold Spring Harb Perspect Biol. 2009;1:a002923 pubmed publisher
    ..This review assesses a variety of cell-cell junction-to-nuclear signaling pathways, and outlines interesting areas for further study...
  40. Garrod D, Berika M, Bardsley W, Holmes D, Tabernero L. Hyper-adhesion in desmosomes: its regulation in wound healing and possible relationship to cadherin crystal structure. J Cell Sci. 2005;118:5743-54 pubmed
    The resistance of tissues to physical stress is dependent upon strong cell-cell adhesion in which desmosomes play a crucial role...
  41. Calkins C, Setzer S, Jennings J, Summers S, Tsunoda K, Amagai M, et al. Desmoglein endocytosis and desmosome disassembly are coordinated responses to pemphigus autoantibodies. J Biol Chem. 2006;281:7623-34 pubmed
    b>Desmosomes are adhesive intercellular junctions prominent in the skin and heart. Loss of desmosome function is associated with severe congenital and acquired disorders characterized by tissue fragility...
  42. Cirillo N, Femiano F, Gombos F, Lanza A. Serum from pemphigus vulgaris reduces desmoglein 3 half-life and perturbs its de novo assembly to desmosomal sites in cultured keratinocytes. FEBS Lett. 2006;580:3276-81 pubmed
    ..Conversely, incorporation of PV IgG-Dsg3 complexes into new forming desmosomes appears perturbed...
  43. Nguyen B, Dusek R, BEAUDRY V, Marinkovich M, Attardi L. Loss of the desmosomal protein perp enhances the phenotypic effects of pemphigus vulgaris autoantibodies. J Invest Dermatol. 2009;129:1710-8 pubmed publisher
    ..Together, our findings highlight the importance of non-cadherin desmosomal proteins in modulating PV phenotypes and provide new insight into Perp's role in the desmosome...
  44. Fressart V, Duthoit G, Donal E, Probst V, Deharo J, Chevalier P, et al. Desmosomal gene analysis in arrhythmogenic right ventricular dysplasia/cardiomyopathy: spectrum of mutations and clinical impact in practice. Europace. 2010;12:861-8 pubmed publisher
    ..We wanted to address the potential impact of genotyping...
  45. Lahtinen A, Lehtonen E, Marjamaa A, Kaartinen M, Helio T, Porthan K, et al. Population-prevalent desmosomal mutations predisposing to arrhythmogenic right ventricular cardiomyopathy. Heart Rhythm. 2011;8:1214-21 pubmed publisher
    ..Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a progressive myocardial disorder caused by mutations of desmosomal cell adhesion proteins. The prevalence of these variants in the general population is unknown...
  46. Brennan D, Peltonen S, Dowling A, Medhat W, Green K, Wahl J, et al. A role for caveolin-1 in desmoglein binding and desmosome dynamics. Oncogene. 2012;31:1636-48 pubmed publisher
  47. Smith W. Guidelines for the diagnosis and management of arrhythmogenic right ventricular cardiomyopathy. Heart Lung Circ. 2011;20:757-60 pubmed publisher
    ..It most commonly presents with palpitations or syncope but sudden death may occur, especially in young males...
  48. Cirillo N, Gombos F, Lanza A. Changes in desmoglein 1 expression and subcellular localization in cultured keratinocytes subjected to anti-desmoglein 1 pemphigus autoimmunity. J Cell Physiol. 2007;210:411-6 pubmed
    ..Furthermore, the present study provides insight into the mechanisms underlying epithelial blistering observed in the skin type of pemphigus...
  49. Waschke J, Spindler V, Bruggeman P, Zillikens D, Schmidt G, Drenckhahn D. Inhibition of Rho A activity causes pemphigus skin blistering. J Cell Biol. 2006;175:721-7 pubmed
    ..This may open the possibility of pemphigus treatment with the epidermal application of Rho A agonists...
  50. Chernyavsky A, Arredondo J, Kitajima Y, Sato Nagai M, Grando S. Desmoglein versus non-desmoglein signaling in pemphigus acantholysis: characterization of novel signaling pathways downstream of pemphigus vulgaris antigens. J Biol Chem. 2007;282:13804-12 pubmed
    ..activation of p38 MAPK is a late signaling step associated with collapse of the cytoskeleton and disassembly of desmosomes caused by upstream events involving Src and EGFRK...
  51. Chen J, Den Z, Koch P. Loss of desmocollin 3 in mice leads to epidermal blistering. J Cell Sci. 2008;121:2844-9 pubmed publisher
    Desmocollin 3 (DSC3) belongs to a subfamily of cadherins and is a major component of desmosomes in keratinocytes of stratified epithelia, such as the epidermis...
  52. Green K, Getsios S, Troyanovsky S, Godsel L. Intercellular junction assembly, dynamics, and homeostasis. Cold Spring Harb Perspect Biol. 2010;2:a000125 pubmed publisher
    ..on assembly of so-called "anchoring" or "adhering" junctions-adherens junctions (AJs) and desmosomes (DSMs), which associate with actin and intermediate filaments, respectively...
  53. Dokuparti M, Pamuru P, Thakkar B, Tanjore R, Nallari P. Etiopathogenesis of arrhythmogenic right ventricular cardiomyopathy. J Hum Genet. 2005;50:375-81 pubmed
    ..desmoplakin (ARVC8) and plakophilin (ARVC9) have prompted the speculation that ARVC is primarily a disease of desmosomes. But identification of TGFbeta-3 for ARVC1 and the role of all these three genes (plakoglobin, desmoplakin and ..
  54. Garrod D, Chidgey M. Desmosome structure, composition and function. Biochim Biophys Acta. 2008;1778:572-87 pubmed
    b>Desmosomes are intercellular junctions of epithelia and cardiac muscle...
  55. Asimaki A, Syrris P, Wichter T, Matthias P, Saffitz J, McKenna W. A novel dominant mutation in plakoglobin causes arrhythmogenic right ventricular cardiomyopathy. Am J Hum Genet. 2007;81:964-73 pubmed
    ..Electron microscopy showed smaller and fewer desmosomes in cells expressing mutant plakoglobin...
  56. Desai B, Harmon R, Green K. Desmosomes at a glance. J Cell Sci. 2009;122:4401-7 pubmed publisher
  57. Franke W. Discovering the molecular components of intercellular junctions--a historical view. Cold Spring Harb Perspect Biol. 2009;1:a003061 pubmed publisher
    ..have been classified and the molecular composition of which has been elucidated in the past three decades: Desmosomes, which connect epithelial and some other cell types, and the almost ubiquitous adherens junctions are based on ..
  58. Mannan T, Jing S, Foroushania S, Fortune F, Wan H. RNAi-mediated inhibition of the desmosomal cadherin (desmoglein 3) impairs epithelial cell proliferation. Cell Prolif. 2011;44:301-10 pubmed publisher
    ..pemphigus vulgaris, where autoantibodies target Dsg3 on keratinocyte surfaces and cause Dsg3 depletion from desmosomes. However, recognition of possible participation of involvement of Dsg3 in cell proliferation remains ..
  59. Gerull B, Heuser A, Wichter T, Paul M, Basson C, McDermott D, et al. Mutations in the desmosomal protein plakophilin-2 are common in arrhythmogenic right ventricular cardiomyopathy. Nat Genet. 2004;36:1162-4 pubmed
    ..In two kindreds with ARVC, disease was incompletely penetrant in most carriers of PKP2 mutations...
  60. Nagasaka T, Nishifuji K, Ota T, Whittock N, Amagai M. Defining the pathogenic involvement of desmoglein 4 in pemphigus and staphylococcal scalded skin syndrome. J Clin Invest. 2004;114:1484-92 pubmed
    ..These findings suggest that Dsg4 may play a role other than adhesion and that the cross-reactivity of desmoglein autoantibodies should be factored into the framework of future studies of autoimmune mechanisms in pemphigus...
  61. Wahl J. A role for plakophilin-1 in the initiation of desmosome assembly. J Cell Biochem. 2005;96:390-403 pubmed
    ..in skin fragility-ectodermal dysplasia syndrome, which is characterized by a reduction in the number and size of desmosomes in the epithelia of affected individuals...
  62. Choi H, Gross J, Pokutta S, Weis W. Interactions of plakoglobin and beta-catenin with desmosomal cadherins: basis of selective exclusion of alpha- and beta-catenin from desmosomes. J Biol Chem. 2009;284:31776-88 pubmed publisher
    ..Plakoglobin, but normally not beta-catenin, is also a structural constituent of desmosomes, where it binds to the cytoplasmic domains of the desmosomal cadherins, desmogleins and desmocollins...
  63. Sobolik Delmaire T, Reddy R, Pashaj A, Roberts B, Wahl J. Plakophilin-1 localizes to the nucleus and interacts with single-stranded DNA. J Invest Dermatol. 2010;130:2638-46 pubmed publisher
    ..These data suggest that in addition to mediating desmosome assembly, the nuclear pool of Pkp can influence cell survival by interactions with DNA...
  64. Gehmlich K, Syrris P, Peskett E, Evans A, Ehler E, Asimaki A, et al. Mechanistic insights into arrhythmogenic right ventricular cardiomyopathy caused by desmocollin-2 mutations. Cardiovasc Res. 2011;90:77-87 pubmed publisher
    ..However, the underlying molecular processes are poorly understood...
  65. Ishida Yamamoto A, Igawa S, Kishibe M. Order and disorder in corneocyte adhesion. J Dermatol. 2011;38:645-54 pubmed publisher
    Epidermal cornified cells are attached to each other with modified desmosomes, namely corneodesmosomes...
  66. Bauce B, Rampazzo A, Basso C, Mazzotti E, Rigato I, Steriotis A, et al. Clinical phenotype and diagnosis of arrhythmogenic right ventricular cardiomyopathy in pediatric patients carrying desmosomal gene mutations. Heart Rhythm. 2011;8:1686-95 pubmed publisher
    ..Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart muscle disease carrying a risk of sudden death. Information about the clinical features during childhood and the age at disease onset is scanty...
  67. Roth W, Kumar V, Beer H, Richter M, Wohlenberg C, Reuter U, et al. Keratin 1 maintains skin integrity and participates in an inflammatory network in skin through interleukin-18. J Cell Sci. 2012;125:5269-79 pubmed publisher
    ..Our data suggest a functional link between KRT1 and human inflammatory skin diseases...
  68. McGrath J, Wessagowit V. Human hair abnormalities resulting from inherited desmosome gene mutations. Keio J Med. 2005;54:72-9 pubmed
    Over the last eight years, several naturally occurring human gene mutations in structural components of desmosomes, cell-cell adhesion junctions found in skin, heart and meninges, have been reported...
  69. Sobolik Delmaire T, Katafiasz D, Wahl J. Carboxyl terminus of Plakophilin-1 recruits it to plasma membrane, whereas amino terminus recruits desmoplakin and promotes desmosome assembly. J Biol Chem. 2006;281:16962-70 pubmed
    ..is the underlying cause of ectodermal dysplasia/skin fragility syndrome, and skin from these patients exhibits desmosomes that are reduced in size and number...
  70. Guttman J, Kazemi P, Lin A, Vogl A, Finlay B. Desmosomes are unaltered during infections by attaching and effacing pathogens. Anat Rec (Hoboken). 2007;290:199-205 pubmed
    ..Based on this, we tested the hypothesis that desmosomes, the only intercellular junctions that interact with intermediate filaments, are also influenced by A/E pathogen ..
  71. Garrod D, Kimura T. Hyper-adhesion: a new concept in cell-cell adhesion. Biochem Soc Trans. 2008;36:195-201 pubmed publisher
    ..We have developed a new concept of cell-cell adhesion termed 'hyper-adhesion', the very strong adhesion adopted by desmosomes. This uniquely desmosomal property accounts for their ability to provide the intercellular links in the ..
  72. Andersen N, Yeaman C. Sec3-containing exocyst complex is required for desmosome assembly in mammalian epithelial cells. Mol Biol Cell. 2010;21:152-64 pubmed publisher
    ..evidence in support of a function for a Sec3-containing Exocyst complex in the assembly or maintenance of desmosomes, adhesive junctions that link intermediate filament networks to sites of strong intercellular adhesion...
  73. den Haan A, Tan B, Zikusoka M, Lladó L, Jain R, Daly A, et al. Comprehensive desmosome mutation analysis in north americans with arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circ Cardiovasc Genet. 2009;2:428-35 pubmed publisher
    ..Compared with those without a desmosome gene mutation, individuals with a desmosome gene mutation had earlier-onset ARVD/C and were more likely to have ventricular tachycardia. ..
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    b>Desmosomes are intercellular junctions that tether intermediate filaments to the plasma membrane. Desmogleins and desmocollins, members of the cadherin superfamily, mediate adhesion at desmosomes...
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    ..However, the role of desmosomes - adhesion complexes that are related to adherens junctions - in carcinogenesis has remained elusive...
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    b>Desmosomes are cell-cell adhesion structures that integrate cytoskeletal networks. In addition to binding intermediate filaments, the desmosomal protein desmoplakin (DP) regulates microtubule reorganization in the epidermis...
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    ..b>Desmosomes are prominent adhesive structures found in a variety of epithelial tissues that are believed to inhibit cell ..
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    b>Desmosomes are adhesive intercellular junctions that mechanically integrate adjacent cells by coupling adhesive interactions mediated by desmosomal cadherins to the intermediate filament cytoskeletal network...
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    ..These human diseases reveal key roles for desmosomes in maintaining tissue integrity, but also suggest functions for desmosomal components in signal transduction ..
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    b>Desmosomes in tissues are resistant to disruption by chelation of extracellular calcium...
  81. Yamamoto Y, Aoyama Y, Shu E, Tsunoda K, Amagai M, Kitajima Y. Anti-desmoglein 3 (Dsg3) monoclonal antibodies deplete desmosomes of Dsg3 and differ in their Dsg3-depleting activities related to pathogenicity. J Biol Chem. 2007;282:17866-76 pubmed
    ..keratinocytes and the presence of autoantibody against desmoglein 3 (Dsg3), which provides adhesive integrity to desmosomes between adjacent keratinocytes...
  82. Chidgey M, Dawson C. Desmosomes: a role in cancer?. Br J Cancer. 2007;96:1783-7 pubmed
    Much evidence now attests to the importance of desmosomes and their constituents in cancer...
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    b>Desmosomes are a complex assembly of protein molecules that form at the cell surface and mediate cell-cell adhesion...
  84. Felder E, Siebenbrunner M, Busch T, Fois G, Miklavc P, Walther P, et al. Mechanical strain of alveolar type II cells in culture: changes in the transcellular cytokeratin network and adaptations. Am J Physiol Lung Cell Mol Physiol. 2008;295:L849-57 pubmed publisher
    ..out at 10% increase in cell surface area (CSA) and that this is accompanied by a widened extracellular gap of desmosomes-the insertion points of CK fibers...
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    ..Development of a prospectively validated risk-stratification algorithm for the full disease spectrum remains the foremost clinical challenge...
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    ..This and the intricate control mechanisms in the stratum corneum that bring about its full and proper structural maturity will be reviewed...
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    ..Thus, the gene coding for desmin appears to be a novel ARVC gene, which should be included in molecular genetic screening of ARVC patients...
  89. Berkowitz P, Hu P, Liu Z, Diaz L, Enghild J, Chua M, et al. Desmosome signaling. Inhibition of p38MAPK prevents pemphigus vulgaris IgG-induced cytoskeleton reorganization. J Biol Chem. 2005;280:23778-84 pubmed
    ..Targeting desmosome signaling via inhibition of p38MAPK and HSP27 phosphorylation may provide novel treatments for PV and other desmosome-associated blistering diseases...
  90. Ihrie R, Attardi L. A new Perp in the lineup: linking p63 and desmosomal adhesion. Cell Cycle. 2005;4:873-6 pubmed
    ..Perp's position downstream of p63 and p53, as well as its essential role in normal desmosome function, suggest that it, like other adhesion proteins, may be a target for mutation in human blistering diseases or cancer...
  91. Preston A, Lindsay L, Murphy C. Desmosomes in uterine epithelial cells decrease at the time of implantation: an ultrastructural and morphometric study. J Morphol. 2006;267:103-8 pubmed
    ..b>Desmosomes, which are part of the lateral junctional complex, function in cell-to-cell adhesion, and are therefore likely ..
  92. Loranger A, Gilbert S, Brouard J, Magin T, Marceau N. Keratin 8 modulation of desmoplakin deposition at desmosomes in hepatocytes. Exp Cell Res. 2006;312:4108-19 pubmed
    Keratins, the intermediate filament proteins of epithelial cells, connect to desmosomes, the cell-cell adhesion structures at the surface membrane...