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desmosome wikipedia jump to content main menu main menu move to sidebar hide navigation main page contents current events random article about wikipedia contact us contribute help learn to edit community portal recent changes upload file special pages search search appearance donate create account log in personal tools donate create account log in contents move to sidebar hide top 1 structure 2 clinical significance toggle clinical significance subsection 2 1 arrhythmogenic cardiomyopathy 2 2 blisters 3 history 4 see also 5 references 6 external links toggle the table of contents desmosome 32 languages العربية български bosanski català čeština deutsch español euskara فارسی suomi français galego hrvatski bahasa indonesia italiano 日本語 қазақша nederlands polski português română русский srpskohrvatski српскохрватски simple english slovenščina српски srpski ไทย türkçe українська oʻzbekcha ўзбекча tiếng việt 中文 edit links article talk english read edit view history tools tools move to sidebar hide actions read edit view history general what links here related changes upload file permanent link page information cite this page get shortened url switch to legacy parser print export download as pdf printable version in other projects wikimedia commons wikidata item appearance move to sidebar hide from wikipedia the free encyclopedia cell junction involved in cell to cell adhesion desmosome a desmosome the extracellular core region containing desmocollin and desmoglein and the plaque contain desmoplakin which attaches to keratin intermediate filaments desmosome is a intercellular junction in animal cell details identifiers latin desmosoma macula adhaerens mesh d003896 th h1 00 01 1 02015 fma 67412 anatomical terminology edit on wikidata a desmosome ˈ d ɛ z m ə ˌ s oʊ m 1 2 binding body also known as a macula adherens plural maculae adherentes latin for adhering spot is a cell structure specialized for cell to cell adhesion a type of junctional complex they are localized spot like adhesions randomly arranged on the lateral sides of plasma membranes desmosomes are one of the stronger cell to cell adhesion types and are found in tissue that experience intense mechanical stress such as cardiac muscle tissue bladder tissue gastrointestinal mucosa and epithelia 3 structure edit desmosomes are composed of desmosome intermediate filament complexes difcs a network of cadherin proteins linker proteins and intermediate filaments 4 the difcs can be broken into three regions the extracellular core region desmoglea the outer dense plaque odp and the inner dense plaque idp 3 the extracellular core region approximately 34 nm in length contains desmoglein and desmocollin which are in the cadherin family of cell adhesion proteins both have five extracellular domains and have calcium binding motifs extracellular calcium helps form the cadherin adhesion by allowing the cadherin extracellular domain on desmoglein and desmocollin to become rigid 5 they bind to each other via heterophilic interactions in the extracellular space near their n termini in contrast with the homophilic binding characteristic of other cadherins 6 desmoglein and desmocollin have a single pass transmembrane region plus an intracellular anchor to secure its position in the cell membrane desmogleins and the desmocollin isoform dsc a contain an intracellular cadherin domain which binds to plakoglobin 3 the outer dense plaque which is about 15 20 nm in length contains the intracellular ends of desmocollin and desmoglein the n terminus side of desmoplakin and the armadillo family of mediatory proteins plakoglobin and plakophilin 3 armadillo proteins are involved in mediating attachment to intracellular filaments and cell membrane proteins armadillo proteins consist of β catenin p120 catenin plakoglobin γ catenin and plakophilins 1 3 in desmosomes plakoglobin and plakophilin help to anchor desmoplakin and keratin filaments to the desmosome structure plakoglobin has 12 arm repeats with a head and tail structure plakophilins have 9 arm repeats and exist in two isoforms a shorter a form and longer b form citation needed the inner dense plaque also about 15 20 nm in length contains the c terminus end of desmoplakin and their attachment to keratin intermediate filaments desmoplakin is the most abundant part of the desmosome 7 as it operates as the mediator between the cadherin proteins in the plasma membrane and the keratin filaments desmoplakin has two isoforms that differ in the length of their middle rod domain all desmoplakins have an n terminal head a c tail consisting of three plakin repeats and a glycine serine arginine rich domain gsr at the c end citation needed clinical significance edit arrhythmogenic cardiomyopathy edit mutations within the desmosome are the main cause of arrhythmogenic cardiomyopathy acm a life threatening disease caused by mutations usually in desmoglein 2 but sometimes in desmocollin 2 it often afflicts individuals between 20 and 50 years and has been publicly known as a cause of death in young athletes although the majority of sudden deaths do not occur in close connection to physical activity the current incidence within the population is accepted as 1 10 000 however it is thought that 1 200 may have a mutation that may predispose to acm 8 symptoms of acm include fainting shortness of breath and heart palpitations and the condition is treated by implanting a small defibrillator device blisters edit blistering diseases such as pemphigus vulgaris pv and pemphigus foliaceus pf are autoimmune diseases in which auto antibodies target desmogleins pv is caused by circulating autoantibodies igg that target dsg3 desmoglein 3 and sometimes dsg1 pv is manifested by suprabasal acantholysis or blisters in the mucous membrane and blisters in the epidermis pf patients have autoantibodies that target dsg1 with superficial blisters on the epidermis with no mucous membrane issues both diseases result in a loss of keratinocyte adhesion pemphigus can also be caused by a bacterial infection bullous impetigo is an infection caused by a staphylococcus bacterium that releases a toxin that cleaves the dsg1 extracellular domain citation needed similar symptoms occur with hailey hailey disease though the cause is not autoimmune but genetic a haploinsufficiency of the atp2c1 gene located on chromosome 3 which encodes the protein hspca1 causes malformation of the desmosomes desmoglein 1 haploinsufficiency leads to striate palmoplantar keratoderma a disease which causes extreme thickening of the epidermis loss of desmoglein 4 leads to defective hair follicle differentiation 3 epidermolysis bullosa simplex is an epidermal blistering disease caused by mutations in genes coding for keratin 5 and 14 which attach to desmoplakin this disease manifests as rupture of the basal epidermis when stress is applied ectodermal dysplasia or skin fragility syndrome is caused by plakophilin 1 mutations this is manifested by detachment of intermediate filaments and desmoplakin from the desmosome 4 history edit the desmosome was first discovered by giulio bizzozero an italian pathologist 3 he named these dense nodules the nodes of bizzozero in 1920 the term desmosome was originated by josef schaffer the first combining form desmo neo latin from greek desmos bond carries meaning of binding or bonding things together combined with some which comes from soma body it thus makes a desmosome a binding body see also edit arrhythmogenic right ventricular cardiomyopathy epidermolysis bullosa hemidesmosome references edit desmosome lexico uk english dictionary oxford university press archived from the original on 2020 01 27 desmosome merriam webster com dictionary merriam webster oclc 1032680871 retrieved 2016 01 21 1 2 3 4 5 6 delva emmanuella tucker dana k kowalczyk andrew p august 2009 the desmosome cold spring harbor perspectives in biology 1 2 a002543 doi 10 1101 cshperspect a002543 issn 1943 0264 pmc 2742091 pmid 20066089 1 2 garrod david chidgey martyn 2008 03 01 desmosome structure composition and function biochimica et biophysica acta bba biomembranes apical junctional complexes part i 1778 3 572 587 doi 10 1016 j bbamem 2007 07 014 pmid 17854763 pokutta sabine weis william i 2007 structure and mechanism of cadherins and catenins in cell cell contacts annual review of cell and developmental biology 23 237 261 doi 10 1146 annurev cellbio 22 010305 104241 issn 1081 0706 pmid 17539752 harrison oliver j brasch julia lasso gorka katsamba phinikoula s ahlsen goran honig barry shapiro lawrence 2016 structural basis of adhesive binding by desmocollins and desmogleins pnas 113 26 7160 7165 bibcode 2016pnas 113 7160h doi 10 1073 pnas 1606272113 pmc 4932976 pmid 27298358 mueller h franke w w 1983 02 05 biochemical and immunological characterization of desmoplakins i and ii the major polypeptides of the desmosomal plaque journal of molecular biology 163 4 647 671 doi 10 1016 0022 2836 83 90116 x issn 0022 2836 pmid 6341602 lahtinen am lehtonen e marjamaa a kaartinen m heliö t porthan k oikarinen l toivonen l swan h jula a peltonen l palotie a salomaa v kontula k 2011 population prevalent desmosomal mutations predisposing to arrhythmogenic right ventricular cardiomyopathy heart rhythm 8 8 1214 21 doi 10 1016 j hrthm 2011 03 015 pmid 21397041 external links edit wikimedia commons has media related to desmosomes meded at loyola histo practical epithelium hp1 13 html histology image 20502loa histology learning system at boston university ultrastructure of the cell microvillous border junctional complex of absorptive epithelium histology image 20604loa histology learning system at boston university ultrastructure of the cell microvillous border and junctional complex desmosomes and zonula adhaerens histology image 22502loa histology learning system at boston university ultrastructure of the cell cardiac muscle intercalated disk v t e proteins of epithelium lateral cell cell cell adhesion molecules adherens junction cadherin desmosome desmoglein ion channels gap junction connexon connexin cytoskeleton desmosome desmoplakin plakoglobin tonofibril other membrane proteins tight junction claudin occludin marveld2 basal cell matrix basal lamina hemidesmosome tonofibril focal adhesion costamere apical cilia kinocilium microvilli stereocilia strc authority control databases gnd retrieved from https en wikipedia org w index php title desmosome oldid 1372974819 categories cell anatomy desmosomes hidden categories articles with short description short description is different from wikidata all articles with unsourced statements articles with unsourced statements from march 2023 commons category link is on wikidata this page was last edited on 3 september 2026 at 07 41 utc page was rendered with parsoid text is available under the creative commons attribution sharealike 4 0 license additional terms may apply by using this site you agree to the terms of use and privacy policy 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