{{cs1 config|name-list-style=vanc}} {{Short description|Protein found in humans}} {{Infobox_gene}} thumb|350px|Cell adhesion in desmosomes '''Desmoplakin''' is a protein in humans that is encoded by the ''DSP'' gene.<ref name="pmid1889810">{{cite journal | vauthors = Arnemann J, Spurr NK, Wheeler GN, Parker AE, Buxton RS | title = Chromosomal assignment of the human genes coding for the major proteins of the desmosome junction, desmoglein DGI (DSG), desmocollins DGII/III (DSC), desmoplakins DPI/II (DSP), and plakoglobin DPIII (JUP) | journal = Genomics | volume = 10 | issue = 3 | pages = 640–5 | date = October 1991 | pmid = 1889810 | doi = 10.1016/0888-7543(91)90446-L }}</ref><ref name="entrez">{{cite web | title = Entrez Gene: DSP desmoplakin| url = https://www.ncbi.nlm.nih.gov/gene?Db=gene&Cmd=ShowDetailView&TermToSearch=1832}}</ref><ref name="pmid8769422">{{cite journal | vauthors = Bornslaeger EA, Corcoran CM, Stappenbeck TS, Green KJ | title = Breaking the connection: displacement of the desmosomal plaque protein desmoplakin from cell-cell interfaces disrupts anchorage of intermediate filament bundles and alters intercellular junction assembly | journal = J. Cell Biol. | volume = 134 | issue = 4 | pages = 985–1001 | date = August 1996 | pmid = 8769422 | pmc = 2120955 | doi = 10.1083/jcb.134.4.985 }}</ref> Desmoplakin is a critical component of desmosome structures in cardiac muscle and epidermal cells, which function to maintain the structural integrity at adjacent cell contacts. In cardiac muscle, desmoplakin is localized to intercalated discs which mechanically couple cardiac cells to function in a coordinated syncytial structure. Mutations in desmoplakin have been shown to play a role in dilated cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy, where it may present with acute myocardial injury;<ref>{{cite journal | vauthors = Schoonvelde SA, Hirsch A, Yap SC |title=Desmoplakin cardiomyopathy—an inherited cardiomyopathy presenting with recurrent episodes of acute myocardial injury |journal=Neth Heart J |date=2022 |volume=31 |issue=7–8 |pages=282–286 |doi=10.1007/s12471-022-01735-2|pmid=36434384 |pmc=10400739 |doi-access=free }}</ref><ref>{{cite journal | vauthors = Scheel PJ, Murray B, Tichnell C |title=Arrhythmogenic right ventricular cardiomyopathy presenting as clinical myocarditis in women. |journal=Am J Cardiol |date=2021 |volume=145 |pages=128–134 |doi=10.1016/j.amjcard.2020.12.090|pmid=33460606 |s2cid=231641047 }}</ref> striate palmoplantar keratoderma, Carvajal syndrome and paraneoplastic pemphigus.
==Structure== Desmoplakin exists as two predominant isoforms; the first, known as "DPII", has molecular weight 260.0 kDa (2272 amino acids) and the second, known as "DPI", has molecular weight 332.0 kDa (2871 amino acids).<ref>{{cite web|title=Protein sequence of human desmoplakin (Uniprot ID: P15924)|url=http://www.heartproteome.org/copa/ProteinInfo.aspx?QType=Protein%20ID&QValue=P15924|website=Cardiac Organellar Protein Atlas Knowledgabase (COPaKB)|access-date=26 June 2015|archive-url=https://web.archive.org/web/20150627034108/http://www.heartproteome.org/copa/ProteinInfo.aspx?QType=Protein%20ID&QValue=P15924|archive-date=27 June 2015|url-status=dead}}</ref><ref>{{cite web|title=Protein sequence of human desmoplakin (Uniprot ID: P15924-2)|url=http://www.heartproteome.org/copa/ProteinInfo.aspx?QType=Protein%20ID&QValue=P15924-2|website=Cardiac Organellar Protein Atlas Knowledgebase (COPaKB)|access-date=26 June 2015|archive-url=https://web.archive.org/web/20150627022313/http://www.heartproteome.org/copa/ProteinInfo.aspx?QType=Protein%20ID&QValue=P15924-2|archive-date=27 June 2015|url-status=dead}}</ref> These isoforms are identical except for the shorter rod domain in DPII. DPI is the predominant isoform expressed in cardiac muscle.<ref>{{cite journal | vauthors = Al-Jassar C, Bikker H, Overduin M, Chidgey M | title = Mechanistic basis of desmosome-targeted diseases | journal = Journal of Molecular Biology | volume = 425 | issue = 21 | pages = 4006–22 | date = Nov 2013 | pmid = 23911551 | doi = 10.1016/j.jmb.2013.07.035 | pmc=3807649}}</ref> The ''DSP'' gene is located on chromosome 6p24.3, containing 24 exons and spanning approximately 45 kDa of genomic DNA.<ref name="ReferenceA">{{cite journal | vauthors = Green KJ, Parry DA, Steinert PM, Virata ML, Wagner RM, Angst BD, Nilles LA | title = Structure of the human desmoplakins. Implications for function in the desmosomal plaque | journal = The Journal of Biological Chemistry | volume = 265 | issue = 5 | pages = 2603–12 | date = Feb 1990 | doi = 10.1016/S0021-9258(19)39844-8 | pmid = 1689290 | doi-access = free }}</ref> Desmoplakin is a large desmosomal plaque protein that homodimerizes and adopts a dumbbell-shaped conformation.<ref name="ReferenceA"/> The N-terminal globular head domain of desmoplakin is composed of a series of alpha helical bundles, and is required for both the localization to the desmosome and interaction with the N-terminal region of plakophilin 1 and plakoglobin as well as desmocollin and desmoglein.<ref name = "Smith_1998">{{cite journal | vauthors = Smith EA, Fuchs E | title = Defining the interactions between intermediate filaments and desmosomes | journal = J. Cell Biol. | volume = 141 | issue = 5 | pages = 1229–41 | year = 1998 | pmid = 9606214 | pmc = 2137181 | doi = 10.1083/jcb.141.5.1229 }}</ref> This is further sub divided into a region called the "Plakin domain" made up of six spectrin repeat domains separated by SH3 domain.<ref>{{cite journal | vauthors = Jefferson JJ, Ciatto C, Shapiro L, Liem RK | title = Structural analysis of the plakin domain of bullous pemphigoid antigen1 (BPAG1) suggests that plakins are members of the spectrin superfamily | journal = Journal of Molecular Biology | volume = 366 | issue = 1 | pages = 244–57 | date = Feb 2007 | pmid = 17161423 | doi = 10.1016/j.jmb.2006.11.036 | pmc=1850962}}</ref> A crystal structure of part of the plakin domain has been resolved,<ref>{{cite journal | vauthors = Choi HJ, Weis WI | title = Crystal structure of a rigid four-spectrin-repeat fragment of the human desmoplakin plakin domain | journal = J. Mol. Biol. | volume = 409 | issue = 5 | pages = 800–12 | year = 2011 | pmid = 21536047 | pmc = 3107870 | doi = 10.1016/j.jmb.2011.04.046 }}</ref> while the entire plakin domain has been elucidated using small angle X-ray scattering which revealed a non-linear structure, an unexpected result considering spectrin repeats are observed in linear orientations.<ref name = "Al-Jassar_2011">{{cite journal | vauthors = Al-Jassar C, Knowles T, Jeeves M, Kami K, Behr E, Bikker H, Overduin M, Chidgey M | title = The nonlinear structure of the desmoplakin plakin domain and the effects of cardiomyopathy-linked mutations | journal = J. Mol. Biol. | volume = 411 | issue = 5 | pages = 1049–61 | year = 2011 | pmid = 21756917 | doi = 10.1016/j.jmb.2011.06.047 }}</ref> The C-terminal region of desmoplakin is composed of three plakin repeat domains, termed A, B and C, which are essential for coalignment and binding of intermediate filaments.<ref name = "Smith_1998" /><ref>{{cite journal | vauthors = Choi HJ, Park-Snyder S, Pascoe LT, Green KJ, Weis WI | title = Structures of two intermediate filament-binding fragments of desmoplakin reveal a unique repeat motif structure | journal = Nature Structural Biology | volume = 9 | issue = 8 | pages = 612–20 | date = Aug 2002 | pmid = 12101406 | doi = 10.1038/nsb818 | s2cid = 10611026 }}</ref><ref>{{cite journal | vauthors = Stappenbeck TS, Bornslaeger EA, Corcoran CM, Luu HH, Virata ML, Green KJ | title = Functional analysis of desmoplakin domains: specification of the interaction with keratin versus vimentin intermediate filament networks | journal = The Journal of Cell Biology | volume = 123 | issue = 3 | pages = 691–705 | date = Nov 1993 | pmid = 7693716 | doi=10.1083/jcb.123.3.691 | pmc=2200123}}</ref> Located at the most distal C-terminus of desmoplakin is a region rich in glycine–serine–arginine; it has been demonstrated that serine phosphorylation of this domain may modify desmoplakin-intermediate filament interactions.<ref>{{cite journal | vauthors = Stappenbeck TS, Lamb JA, Corcoran CM, Green KJ | title = Phosphorylation of the desmoplakin COOH terminus negatively regulates its interaction with keratin intermediate filament networks | journal = The Journal of Biological Chemistry | volume = 269 | issue = 47 | pages = 29351–4 | date = Nov 1994 | doi = 10.1016/S0021-9258(18)43881-1 | pmid = 7525582 | doi-access = free }}</ref> In the mid-region of desmoplakin, a coiled-coil rod domain is responsible for homodimerization.<ref name="Garrod D 2007">{{cite journal | vauthors = Garrod D, Chidgey M | title = Desmosome structure, composition and function | journal = Biochimica et Biophysica Acta (BBA) - Biomembranes | volume = 1778 | issue = 3 | pages = 572–87 | date = Mar 2008 | pmid = 17854763 | doi = 10.1016/j.bbamem.2007.07.014 | doi-access = free }}</ref>
== Function == Desmosomes are intercellular junctions that tightly link adjacent cells. Desmoplakin is an obligate component of functional desmosomes that anchors intermediate filaments to desmosomal plaques. In cardiomyocytes, desmoplakin forms desmosomal plaques with the intermediate filament desmin, whereas in endothelial cells cytokeratin type intermediate filaments are recruited, and vimentin in arachnoid and follicular dendritic cell types.<ref name="Garrod D 2007"/><ref>{{cite journal | vauthors = Kartenbeck J, Schwechheimer K, Moll R, Franke WW | title = Attachment of vimentin filaments to desmosomal plaques in human meningiomal cells and arachnoidal tissue | journal = The Journal of Cell Biology | volume = 98 | issue = 3 | pages = 1072–81 | date = Mar 1984 | pmid = 6365927 | doi=10.1083/jcb.98.3.1072 | pmc=2113124}}</ref> Both types of intermediate filaments attach in a lateral fashion to desmoplakin to form the plaque.<ref>{{cite journal | vauthors = Kartenbeck J, Franke WW, Moser JG, Stoffels U | title = Specific attachment of desmin filaments to desmosomal plaques in cardiac myocytes | journal = The EMBO Journal | volume = 2 | issue = 5 | pages = 735–42 | date = 1983 | pmid = 6416832 | pmc=555178| doi = 10.1002/j.1460-2075.1983.tb01493.x }}</ref> In cardiac muscle, desmoplakin is localized to desmosomes in intercalated discs. Desmoplakin isoform DPI is highly expressed and is thought to play a role in both the assembly and stabilization of desmosomes; its role is critical, as desmoplakin knockout mice display embryonic lethality.<ref>{{cite journal | vauthors = Gallicano GI, Kouklis P, Bauer C, Yin M, Vasioukhin V, Degenstein L, Fuchs E | title = Desmoplakin is required early in development for assembly of desmosomes and cytoskeletal linkage | journal = The Journal of Cell Biology | volume = 143 | issue = 7 | pages = 2009–22 | date = Dec 1998 | pmid = 9864371 | doi=10.1083/jcb.143.7.2009 | pmc=2175222}}</ref> In mice overexpressing a C-terminal mutated desmoplakin protein, desmoplakin binding to desmin is disrupted in cardiac muscle and hearts display abnormal intercalated disc formation and structure.<ref name="ReferenceB">{{cite journal | vauthors = Yang Z, Bowles NE, Scherer SE, Taylor MD, Kearney DL, Ge S, Nadvoretskiy VV, DeFreitas G, Carabello B, Brandon LI, Godsel LM, Green KJ, Saffitz JE, Li H, Danieli GA, Calkins H, Marcus F, Towbin JA | title = Desmosomal dysfunction due to mutations in desmoplakin causes arrhythmogenic right ventricular dysplasia/cardiomyopathy | journal = Circulation Research | volume = 99 | issue = 6 | pages = 646–55 | date = Sep 2006 | pmid = 16917092 | doi = 10.1161/01.RES.0000241482.19382.c6 | doi-access = free }}</ref> Much has been learned regarding desmoplakin function from mutations in patients with arrhythmogenic right ventricular cardiomyopathy, where mutations in specific binding domains alter desmoplakin binding to plakoglobin or desmin and result in cell death and dysfunction.<ref>{{cite journal | vauthors = Awad MM, Calkins H, Judge DP | title = Mechanisms of disease: molecular genetics of arrhythmogenic right ventricular dysplasia/cardiomyopathy | journal = Nature Clinical Practice Cardiovascular Medicine | volume = 5 | issue = 5 | pages = 258–67 | date = May 2008 | pmid = 18382419 | doi = 10.1038/ncpcardio1182 | pmc=2822988}}</ref>
==Clinical significance== Mutations in this gene are the cause of several cardiomyopathies, including dilated cardiomyopathy<ref name="ReferenceC">{{cite journal | vauthors = Norgett EE, Hatsell SJ, Carvajal-Huerta L, Cabezas JC, Common J, Purkis PE, Whittock N, Leigh IM, Stevens HP, Kelsell DP | title = Recessive mutation in desmoplakin disrupts desmoplakin-intermediate filament interactions and causes dilated cardiomyopathy, woolly hair and keratoderma | journal = Human Molecular Genetics | volume = 9 | issue = 18 | pages = 2761–6 | date = Nov 2000 | pmid = 11063735 | doi=10.1093/hmg/9.18.2761| doi-access = free }}</ref><ref>{{cite journal | vauthors = Carvajal-Huerta L | title = Epidermolytic palmoplantar keratoderma with woolly hair and dilated cardiomyopathy | journal = Journal of the American Academy of Dermatology | volume = 39 | issue = 3 | pages = 418–21 | date = Sep 1998 | pmid = 9738775 | doi=10.1016/s0190-9622(98)70317-2}}</ref> and arrhythmogenic right ventricular cardiomyopathy.<ref name="ReferenceB"/><ref>{{cite journal | vauthors = Rampazzo A, Nava A, Malacrida S, Beffagna G, Bauce B, Rossi V, Zimbello R, Simionati B, Basso C, Thiene G, Towbin JA, Danieli GA | title = Mutation in human desmoplakin domain binding to plakoglobin causes a dominant form of arrhythmogenic right ventricular cardiomyopathy | journal = American Journal of Human Genetics | volume = 71 | issue = 5 | pages = 1200–6 | date = Nov 2002 | pmid = 12373648 | doi = 10.1086/344208 | pmc=385098}}</ref><ref>{{cite journal | vauthors = Alcalai R, Metzger S, Rosenheck S, Meiner V, Chajek-Shaul T | title = A recessive mutation in desmoplakin causes arrhythmogenic right ventricular dysplasia, skin disorder, and woolly hair | journal = Journal of the American College of Cardiology | volume = 42 | issue = 2 | pages = 319–27 | date = Jul 2003 | pmid = 12875771 | doi=10.1016/s0735-1097(03)00628-4| doi-access = free }}</ref><ref name="Uzumcu A 2005">{{cite journal | vauthors = Uzumcu A, Norgett EE, Dindar A, Uyguner O, Nisli K, Kayserili H, Sahin SE, Dupont E, Severs NJ, Leigh IM, Yuksel-Apak M, Kelsell DP, Wollnik B | title = Loss of desmoplakin isoform I causes early onset cardiomyopathy and heart failure in a Naxos-like syndrome | journal = Journal of Medical Genetics | volume = 43 | issue = 2 | pages = e5 | date = Feb 2006 | pmid = 16467215 | doi = 10.1136/jmg.2005.032904 | pmc=2564645}}</ref><ref>{{cite journal | vauthors = van der Zwaag PA, Jongbloed JD, van den Berg MP, van der Smagt JJ, Jongbloed R, Bikker H, Hofstra RM, van Tintelen JP | title = A genetic variants database for arrhythmogenic right ventricular dysplasia/cardiomyopathy | journal = Human Mutation | volume = 30 | issue = 9 | pages = 1278–83 | date = Sep 2009 | pmid = 19569224 | doi = 10.1002/humu.21064 | s2cid = 7138963 | doi-access = free }}</ref><ref name = "Al-Jassar_2011" /> The presence of pathogenic mutations in this gene has been associated with episodes of acute myocardial injury, which may mimic episodes of myocarditis.<ref>{{cite journal | vauthors = Schoonvelde SA, Hirsch A, Yap SC |title=Desmoplakin cardiomyopathy—an inherited cardiomyopathy presenting with recurrent episodes of acute myocardial injury |journal=Neth Heart J |date=2022 |volume=31 |issue=7–8 |pages=282–286 |doi=10.1007/s12471-022-01735-2|pmid=36434384 |pmc=10400739 |doi-access=free }}</ref><ref>{{cite journal | vauthors = Scheel PJ, Murray B, Tichnell C |title=Arrhythmogenic right ventricular cardiomyopathy presenting as clinical myocarditis in women. |journal=Am J Cardiol |date=2021 |volume=145 |pages=128–134 |doi=10.1016/j.amjcard.2020.12.090|pmid=33460606 |s2cid=231641047 }}</ref> Mutations in ''DSP'' have also been associated with striate palmoplantar keratoderma.<ref name="ReferenceC"/><ref name="Uzumcu A 2005"/><ref>{{cite journal | vauthors = Armstrong DK, McKenna KE, Purkis PE, Green KJ, Eady RA, Leigh IM, Hughes AE | title = Haploinsufficiency of desmoplakin causes a striate subtype of palmoplantar keratoderma | journal = Human Molecular Genetics | volume = 8 | issue = 1 | pages = 143–8 | date = Jan 1999 | pmid = 9887343 | doi=10.1093/hmg/8.1.143| doi-access = free }}</ref><ref>{{cite journal | vauthors = Whittock NV, Ashton GH, Dopping-Hepenstal PJ, Gratian MJ, Keane FM, Eady RA, McGrath JA | title = Striate palmoplantar keratoderma resulting from desmoplakin haploinsufficiency | journal = The Journal of Investigative Dermatology | volume = 113 | issue = 6 | pages = 940–6 | date = Dec 1999 | pmid = 10594734 | doi = 10.1046/j.1523-1747.1999.00783.x | doi-access = free }}</ref><ref>{{cite journal | vauthors = Whittock NV, Wan H, Morley SM, Garzon MC, Kristal L, Hyde P, McLean WH, Pulkkinen L, Uitto J, Christiano AM, Eady RA, McGrath JA | title = Compound heterozygosity for non-sense and mis-sense mutations in desmoplakin underlies skin fragility/woolly hair syndrome | journal = The Journal of Investigative Dermatology | volume = 118 | issue = 2 | pages = 232–8 | date = Feb 2002 | pmid = 11841538 | doi = 10.1046/j.0022-202x.2001.01664.x | doi-access = free }}</ref> Carvajal syndrome results from an autosomal recessive mutation of a frameshift (7901delG) in ''DSP'' that results in a combination of above conditions, including dilated cardiomyopathy, keratoderma and woolly hair.<ref>{{cite journal | vauthors = Carvajal-Huerta L | title = Epidermolytic palmoplantar keratoderma with woolly hair and dilated cardiomyopathy | journal = Journal of the American Academy of Dermatology | volume = 39 | issue = 3 | pages = 418–21 | date = Sep 1998 | pmid = 9738775 | doi=10.1016/s0190-9622(98)70317-2}}</ref> Patients with Carvajal syndrome often suffer from heart failure in teenage years. A case of compound heterozygosity for two ''DSP'' nonsense mutations resulting in lethal acantholytic epidermolysis bullosa has been reported.<ref>{{cite journal | vauthors = Jonkman MF, Pasmooij AM, Pasmans SG, van den Berg MP, Ter Horst HJ, Timmer A, Pas HH | title = Loss of desmoplakin tail causes lethal acantholytic epidermolysis bullosa | journal = American Journal of Human Genetics | volume = 77 | issue = 4 | pages = 653–60 | date = Oct 2005 | pmid = 16175511 | doi = 10.1086/496901 | pmc=1275614}}</ref><ref>{{cite journal | vauthors = McGrath JA, Bolling MC, Jonkman MF | title = Lethal acantholytic epidermolysis bullosa | journal = Dermatologic Clinics | volume = 28 | issue = 1 | pages = 131–5 | date = Jan 2010 | pmid = 19945626 | doi = 10.1016/j.det.2009.10.015 | s2cid = 36535041 }}</ref> Autoantibodies to ''DSP'' are a hallmark of the autoimmune disease paraneoplastic pemphigus.<ref>{{cite journal | vauthors = Anhalt GJ, Kim SC, Stanley JR, Korman NJ, Jabs DA, Kory M, Izumi H, Ratrie H, Mutasim D, Ariss-Abdo L | title = Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia | journal = The New England Journal of Medicine | volume = 323 | issue = 25 | pages = 1729–35 | date = Dec 1990 | pmid = 2247105 | doi = 10.1056/NEJM199012203232503 | doi-access =free }}</ref><ref>{{cite journal | vauthors = Oursler JR, Labib RS, Ariss-Abdo L, Burke T, O'Keefe EJ, Anhalt GJ | title = Human autoantibodies against desmoplakins in paraneoplastic pemphigus | journal = The Journal of Clinical Investigation | volume = 89 | issue = 6 | pages = 1775–82 | date = Jun 1992 | pmid = 1601988 | doi = 10.1172/JCI115781 | pmc=295873}}</ref> Decreased desmoplakin expression has been found in patients with oropharyngeal cancer and breast cancer, which may alter cell-cell adhesion properties and propagate metastasis.<ref>{{cite journal | vauthors = Papagerakis S, Shabana AH, Pollock BH, Papagerakis P, Depondt J, Berdal A | title = Altered desmoplakin expression at transcriptional and protein levels provides prognostic information in human oropharyngeal cancer | journal = Human Pathology | volume = 40 | issue = 9 | pages = 1320–9 | date = Sep 2009 | pmid = 19386346 | doi = 10.1016/j.humpath.2009.02.002 }}</ref><ref>{{cite journal | vauthors = Pang H, Rowan BG, Al-Dhaheri M, Faber LE | title = Epidermal growth factor suppresses induction by progestin of the adhesion protein desmoplakin in T47D breast cancer cells | journal = Breast Cancer Research | volume = 6 | issue = 3 | pages = R239–45 | date = 2004 | pmid = 15084247 | doi = 10.1186/bcr780 | pmc=400677 | doi-access = free }}</ref>
== Interactions == Desmoplakin has been shown to interact with: {{div col|colwidth=20em}} * Desmin,<ref name = pmid9261168/> * Keratin 1,<ref name = pmid9261168/> * PKP1<ref name = pmid10852826>{{cite journal | vauthors = Hofmann I, Mertens C, Brettel M, Nimmrich V, Schnölzer M, Herrmann H | title = Interaction of plakophilins with desmoplakin and intermediate filament proteins: an in vitro analysis | journal = J. Cell Sci. | volume = 113 | issue = 13| pages = 2471–83 | date = July 2000 | doi = 10.1242/jcs.113.13.2471 | pmid = 10852826 }}</ref> * PKP2,<ref name = pmid11790773>{{cite journal | vauthors = Chen X, Bonne S, Hatzfeld M, van Roy F, Green KJ | title = Protein binding and functional characterization of plakophilin 2. Evidence for its diverse roles in desmosomes and beta -catenin signaling | journal = J. Biol. Chem. | volume = 277 | issue = 12 | pages = 10512–22 | date = March 2002 | pmid = 11790773 | doi = 10.1074/jbc.M108765200 | doi-access = free}}</ref> * Plakoglobin,<ref name = pmid9739078>{{cite journal | vauthors = Kowalczyk AP, Navarro P, Dejana E, Bornslaeger EA, Green KJ, Kopp DS, Borgwardt JE | title = VE-cadherin and desmoplakin are assembled into dermal microvascular endothelial intercellular junctions: a pivotal role for plakoglobin in the recruitment of desmoplakin to intercellular junctions | journal = J. Cell Sci. | volume = 111 | issue = 20| pages = 3045–57 | date = October 1998 | doi = 10.1242/jcs.111.20.3045 | pmid = 9739078 }}</ref><ref name = pmid9348293>{{cite journal | vauthors = Kowalczyk AP, Bornslaeger EA, Borgwardt JE, Palka HL, Dhaliwal AS, Corcoran CM, Denning MF, Green KJ | title = The amino-terminal domain of desmoplakin binds to plakoglobin and clusters desmosomal cadherin-plakoglobin complexes | journal = J. Cell Biol. | volume = 139 | issue = 3 | pages = 773–84 | date = November 1997 | pmid = 9348293 | pmc = 2141713 | doi = 10.1083/jcb.139.3.773}}</ref> and * Vimentin.<ref name = pmid9261168>{{cite journal | vauthors = Meng JJ, Bornslaeger EA, Green KJ, Steinert PM, Ip W | title = Two-hybrid analysis reveals fundamental differences in direct interactions between desmoplakin and cell type-specific intermediate filaments | journal = J. Biol. Chem. | volume = 272 | issue = 34 | pages = 21495–503 | date = August 1997 | pmid = 9261168 | doi = 10.1074/jbc.272.34.21495| doi-access = free }}</ref> {{Div col end}}
== See also == * List of target antigens in pemphigus * List of conditions caused by problems with junctional proteins {{Clear}}
== References == {{reflist|33em}}
== Further reading == {{refbegin|33em}} * {{cite journal | vauthors = Presland RB, Dale BA | title = Epithelial structural proteins of the skin and oral cavity: function in health and disease | journal = Crit. Rev. Oral Biol. Med. | volume = 11 | issue = 4 | pages = 383–408 | year = 2000 | pmid = 11132762 | doi = 10.1177/10454411000110040101 }} * {{cite journal | vauthors = Just M, Herbst H, Hummel M, Dürkop H, Tripier D, Stein H, Schuppan D | title = Undulin is a novel member of the fibronectin-tenascin family of extracellular matrix glycoproteins | journal = J. Biol. Chem. | volume = 266 | issue = 26 | pages = 17326–32 | year = 1991 | doi = 10.1016/S0021-9258(19)47377-8 | pmid = 1716629 | doi-access = free }} * {{cite journal | vauthors = Virata ML, Wagner RM, Parry DA, Green KJ | title = Molecular structure of the human desmoplakin I and II amino terminus | journal = Proc. Natl. Acad. Sci. U.S.A. | volume = 89 | issue = 2 | pages = 544–8 | year = 1992 | pmid = 1731325 | pmc = 48275 | doi = 10.1073/pnas.89.2.544 | bibcode = 1992PNAS...89..544V | doi-access = free }} * {{cite journal | vauthors = Green KJ, Parry DA, Steinert PM, Virata ML, Wagner RM, Angst BD, Nilles LA | title = Structure of the human desmoplakins. Implications for function in the desmosomal plaque | journal = J. Biol. Chem. | volume = 265 | issue = 19 | pages = 11406–7 | year = 1990 | doi = 10.1016/S0021-9258(19)38608-9 | pmid = 2391353 | doi-access = free }} * {{cite journal | vauthors = Kouklis PD, Hutton E, Fuchs E | title = Making a connection: direct binding between keratin intermediate filaments and desmosomal proteins | journal = J. Cell Biol. | volume = 127 | issue = 4 | pages = 1049–60 | year = 1994 | pmid = 7525601 | pmc = 2200061 | doi = 10.1083/jcb.127.4.1049 }} * {{cite journal | vauthors = Steinert PM, Marekov LN | title = Direct evidence that involucrin is a major early isopeptide cross-linked component of the keratinocyte cornified cell envelope | journal = J. Biol. Chem. | volume = 272 | issue = 3 | pages = 2021–30 | year = 1997 | pmid = 8999895 | doi = 10.1074/jbc.272.3.2021 | doi-access = free }} * {{cite journal | vauthors = Olavesen MG, Bentley E, Mason RV, Stephens RJ, Ragoussis J | title = Fine mapping of 39 ESTs on human chromosome 6p23-p25 | journal = Genomics | volume = 46 | issue = 2 | pages = 303–6 | year = 1997 | pmid = 9417921 | doi = 10.1006/geno.1997.5032 }} * {{cite journal | vauthors = Marekov LN, Steinert PM | title = Ceramides are bound to structural proteins of the human foreskin epidermal cornified cell envelope | journal = J. Biol. Chem. | volume = 273 | issue = 28 | pages = 17763–70 | year = 1998 | pmid = 9651377 | doi = 10.1074/jbc.273.28.17763 | doi-access = free }} * {{cite journal | vauthors = Suzuki M, Okuyama S, Okamoto S, Shirasuna K, Nakajima T, Hachiya T, Nojima H, Sekiya S, Oda K | title = A novel E2F binding protein with Myc-type HLH motif stimulates E2F-dependent transcription by forming a heterodimer | journal = Oncogene | volume = 17 | issue = 7 | pages = 853–65 | year = 1998 | pmid = 9780002 | doi = 10.1038/sj.onc.1202163 | s2cid = 23588950 | doi-access = }} * {{cite journal | vauthors = Dias Neto E, Correa RG, Verjovski-Almeida S, Briones MR, Nagai MA, da Silva W, Zago MA, Bordin S, Costa FF, Goldman GH, Carvalho AF, Matsukuma A, Baia GS, Simpson DH, Brunstein A, de Oliveira PS, Bucher P, Jongeneel CV, O'Hare MJ, Soares F, Brentani RR, Reis LF, de Souza SJ, Simpson AJ | title = Shotgun sequencing of the human transcriptome with ORF expressed sequence tags | journal = Proc. Natl. Acad. Sci. U.S.A. | volume = 97 | issue = 7 | pages = 3491–6 | year = 2000 | pmid = 10737800 | pmc = 16267 | doi = 10.1073/pnas.97.7.3491 | bibcode = 2000PNAS...97.3491D | doi-access = free }} {{refend}}
== External links == * [https://www.ncbi.nlm.nih.gov/books/NBK1131/ GeneReviews/NCBI/NIH/UW entry on Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy, Autosomal Dominant] * [https://omim.org/search/?index=entry&start=1&limit=10&search=604772+605676+601214+107970+125645+125647+125671+173325+180902+190230+600996+602086+602087+602861+604400+604401+607450+609040+609160+610193+610476+611528+612048+107970+125645+125647+125671+173325+180902+190230+600996+602086+602087+602861+604400+604401+607450+609040+609160+610193+610476+611528+612048&sort=score+desc&field=number OMIM entries on Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy, Autosomal Dominant] * {{MeshName|Desmoplakins}} * {{PDBe-KB2|P15924|Desmoplakin}}
{{PDB Gallery|geneid=1832}} {{Cytoskeletal Proteins}} {{Epithelial tissue}}
Category:Plakins