# Telethonin
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{{Short description|Protein in the human body}}
{{Infobox_gene}}
'''Telethonin''', also known as '''Tcap''', is a [protein](/source/protein) that in humans is encoded by the ''TCAP'' [gene](/source/gene).[{{cite journal | vauthors = Valle G, Faulkner G, De Antoni A, Pacchioni B, Pallavicini A, Pandolfo D, Tiso N, Toppo S, Trevisan S, Lanfranchi G | title = Telethonin, a novel sarcomeric protein of heart and skeletal muscle | journal = FEBS Letters | volume = 415 | issue = 2 | pages = 163–8 | date = Sep 1997 | pmid = 9350988 | doi = 10.1016/S0014-5793(97)01108-3 | s2cid = 1482856 | doi-access = free | hdl = 11577/2469154 | hdl-access = free }}][{{cite journal | vauthors = Gregorio CC, Trombitás K, Centner T, Kolmerer B, Stier G, Kunke K, Suzuki K, Obermayr F, Herrmann B, Granzier H, Sorimachi H, Labeit S | title = The NH2 terminus of titin spans the Z-disc: its interaction with a novel 19-kD ligand (T-cap) is required for sarcomeric integrity | journal = The Journal of Cell Biology | volume = 143 | issue = 4 | pages = 1013–27 | date = Nov 1998 | pmid = 9817758 | pmc = 2132961 | doi = 10.1083/jcb.143.4.1013 }}][{{cite web | title = Entrez Gene: TCAP titin-cap (telethonin)| url = https://www.ncbi.nlm.nih.gov/gene?Db=gene&Cmd=ShowDetailView&TermToSearch=8557}}] Telethonin is expressed in [cardiac](/source/cardiac_muscle) and [skeletal muscle](/source/skeletal_muscle) at [Z-discs](/source/sarcomere) and functions to regulate sarcomere assembly, [T-tubule](/source/T-tubule) function and [apoptosis](/source/apoptosis). Telethonin has been implicated in several diseases, including [limb-girdle muscular dystrophy](/source/limb-girdle_muscular_dystrophy), [hypertrophic cardiomyopathy](/source/hypertrophic_cardiomyopathy), [dilated cardiomyopathy](/source/dilated_cardiomyopathy) and idiopathic [cardiomyopathy](/source/cardiomyopathy).
==Structure==
Telethonin is a 19.0 kDa protein composed of 167 amino acids.[{{cite web|title=Protein Information for the human TCAP gene (Uniprot ID: O15273)|url=http://www.heartproteome.org/copa/ProteinInfo.aspx?QType=Protein%20ID&QValue=O15273|website=COPaKB: Cardiac Organellar Protein Atlas Knowledgebase|access-date=20 June 2015}}{{Dead link|date=August 2025 |bot=InternetArchiveBot |fix-attempted=yes }}]
Telethonin has a unique β-sheet structure, which enables antiparallel association with the [Titin](/source/Titin) Z1-Z2 domains in [cardiac](/source/cardiac_muscle) and [skeletal muscle](/source/skeletal_muscle).[{{cite journal | vauthors = Zou P, Pinotsis N, Lange S, Song YH, Popov A, Mavridis I, Mayans OM, Gautel M, Wilmanns M | title = Palindromic assembly of the giant muscle protein titin in the sarcomeric Z-disk | journal = Nature | volume = 439 | issue = 7073 | pages = 229–33 | date = Jan 2006 | pmid = 16407954 | doi = 10.1038/nature04343 | bibcode = 2006Natur.439..229Z | s2cid = 7509846 | url = http://bib-pubdb1.desy.de/record/80904 }}] Structural analysis of full-length Telethonin with the [N-terminal](/source/N-terminus) region of [Titin](/source/Titin) indicate that the [C-terminus](/source/C-terminus) of Telethonin is critical for the dimerization of two Telethonin/[Titin](/source/Titin) complexes into a higher oligomeric structure.[{{cite journal | vauthors = Pinotsis N, Petoukhov M, Lange S, Svergun D, Zou P, Gautel M, Wilmanns M | title = Evidence for a dimeric assembly of two titin/telethonin complexes induced by the telethonin C-terminus | journal = Journal of Structural Biology | volume = 155 | issue = 2 | pages = 239–50 | date = Aug 2006 | pmid = 16713295 | doi = 10.1016/j.jsb.2006.03.028 }}]
== Function ==
Telethonin expression is developmentally regulated in both [cardiac](/source/cardiac_muscle) and [skeletal muscle](/source/skeletal_muscle) and is thought to be critical to sarcomere assembly.[{{cite journal | vauthors = Mason P, Bayol S, Loughna PT | title = The novel sarcomeric protein telethonin exhibits developmental and functional regulation | journal = Biochemical and Biophysical Research Communications | volume = 257 | issue = 3 | pages = 699–703 | date = Apr 1999 | pmid = 10208846 | doi = 10.1006/bbrc.1999.0531 }}] Telethonin was found to be a late assembling protein only present in mature [myofibrils](/source/myofibrils) at [Z-discs](/source/sarcomere).[{{cite journal | vauthors = White J, Barro MV, Makarenkova HP, Sanger JW, Sanger JM | title = Localization of sarcomeric proteins during myofibril assembly in cultured mouse primary skeletal myotubes | journal = Anatomical Record | volume = 297 | issue = 9 | pages = 1571–84 | date = Sep 2014 | pmid = 25125171 | doi = 10.1002/ar.22981 | pmc=4145531}}]
Telethonin forms a complex with [muscle LIM protein (MLP)](/source/CSRP3) at [sarcomere Z-discs](/source/sarcomere), which constitutes part of the [cardiomyocyte](/source/cardiomyocyte) stretch sensory mechanism.[{{cite journal | vauthors = Knöll R, Hoshijima M, Hoffman HM, Person V, Lorenzen-Schmidt I, Bang ML, Hayashi T, Shiga N, Yasukawa H, Schaper W, McKenna W, Yokoyama M, Schork NJ, Omens JH, McCulloch AD, Kimura A, Gregorio CC, Poller W, Schaper J, Schultheiss HP, Chien KR | title = The cardiac mechanical stretch sensor machinery involves a Z disc complex that is defective in a subset of human dilated cardiomyopathy | journal = Cell | volume = 111 | issue = 7 | pages = 943–55 | date = Dec 2002 | pmid = 12507422 | doi=10.1016/s0092-8674(02)01226-6| s2cid = 15082967 | doi-access = free }}] It has also been shown that Telethonin binds to the beta-subunit of the slow activating component of the [delayed rectifier potassium channel](/source/KvLQT1), MinK, in areas localized to [T-tubule](/source/T-tubule) membranes surrounding [Z-lines](/source/sarcomere) in the inner [myocardium](/source/myocardium).[{{cite journal | vauthors = Furukawa T, Ono Y, Tsuchiya H, Katayama Y, Bang ML, Labeit D, Labeit S, Inagaki N, Gregorio CC | title = Specific interaction of the potassium channel beta-subunit minK with the sarcomeric protein T-cap suggests a T-tubule-myofibril linking system | journal = Journal of Molecular Biology | volume = 313 | issue = 4 | pages = 775–84 | date = Nov 2001 | pmid = 11697903 | doi = 10.1006/jmbi.2001.5053 }}] In addition, Telethonin interacts with the sodium channel [Na(v)1.5](/source/SCN5A), and alters the activation kinetics via doubling the window current.[{{cite journal | vauthors = Mazzone A, Strege PR, Tester DJ, Bernard CE, Faulkner G, De Giorgio R, Makielski JC, Stanghellini V, Gibbons SJ, Ackerman MJ, Farrugia G | title = A mutation in telethonin alters Nav1.5 function | journal = The Journal of Biological Chemistry | volume = 283 | issue = 24 | pages = 16537–44 | date = Jun 2008 | pmid = 18408010 | doi = 10.1074/jbc.M801744200 | pmc=2423252| doi-access = free }}] These data suggest that Telethonin may constitute a mechano-electrical links between [Z-lines](/source/sarcomere) and [T-tubule](/source/T-tubule)s. Further functional evidence for this has come from studies utilizing a Telethonin-knockout mouse (KO), which have shown that Telethonin is involved in [T-tubule](/source/T-tubule) structure and function, as well as apoptosis in the heart. Telethonin KO animals showed preserved [Titin](/source/Titin) anchoring at baseline, and instead showed a profound deficit during [nuclear](/source/Cell_nucleus) biomechanical stress in modulating the turnover of the proapoptotic [p53](/source/p53) protein.[{{cite journal | vauthors = Knöll R, Linke WA, Zou P, Miocic S, Kostin S, Buyandelger B, Ku CH, Neef S, Bug M, Schäfer K, Knöll G, Felkin LE, Wessels J, Toischer K, Hagn F, Kessler H, Didié M, Quentin T, Maier LS, Teucher N, Unsöld B, Schmidt A, Birks EJ, Gunkel S, Lang P, Granzier H, Zimmermann WH, Field LJ, Faulkner G, Dobbelstein M, Barton PJ, Sattler M, Wilmanns M, Chien KR | title = Telethonin deficiency is associated with maladaptation to biomechanical stress in the mammalian heart | journal = Circulation Research | volume = 109 | issue = 7 | pages = 758–69 | date = Sep 2011 | pmid = 21799151 | doi = 10.1161/CIRCRESAHA.111.245787 | pmc=3664427}}] Telethonin KO animals also displayed [calcium](/source/calcium) transient dysynchrony, [T-tubule](/source/T-tubule) loss and depressed [L-type calcium channel](/source/L-type_calcium_channel) function.[{{cite journal | vauthors = Ibrahim M, Siedlecka U, Buyandelger B, Harada M, Rao C, Moshkov A, Bhargava A, Schneider M, Yacoub MH, Gorelik J, Knöll R, Terracciano CM | title = A critical role for Telethonin in regulating t-tubule structure and function in the mammalian heart | journal = Human Molecular Genetics | volume = 22 | issue = 2 | pages = 372–83 | date = Jan 2013 | pmid = 23100327 | doi = 10.1093/hmg/dds434 | pmc=3526164}}]
Telethonin is a substrate of titin kinase,[{{cite journal | vauthors = Candasamy AJ, Haworth RS, Cuello F, Ibrahim M, Aravamudhan S, Krüger M, Holt MR, Terracciano CM, Mayr M, Gautel M, Avkiran M | title = Phosphoregulation of the titin-cap protein telethonin in cardiac myocytes | journal = The Journal of Biological Chemistry | volume = 289 | issue = 3 | pages = 1282–93 | date = Jan 2014 | pmid = 24280220 | doi = 10.1074/jbc.M113.479030 | pmc=3894314| doi-access = free }}] Telethonin, as well as [TNNI3](/source/TNNI3), [MYBPC3](/source/Myosin_binding_protein_C%2C_cardiac) and [MYOM2](/source/MYOM2) are phosphorylated by [PKD](/source/PRKD1) in [cardiomyocytes](/source/cardiomyocytes), and this leads to a reduction in calcium sensitivity of [myofilament](/source/myofilament)s, as well as accelerated [crossbridge](/source/crossbridge) kinetics.[{{cite journal | vauthors = Haworth RS, Cuello F, Herron TJ, Franzen G, Kentish JC, Gautel M, Avkiran M | title = Protein kinase D is a novel mediator of cardiac troponin I phosphorylation and regulates myofilament function | journal = Circulation Research | volume = 95 | issue = 11 | pages = 1091–9 | date = Nov 2004 | pmid = 15514163 | doi = 10.1161/01.RES.0000149299.34793.3c | doi-access = free }}] Bis-phosphorylation of Telethonin specifically at sites [Serine](/source/Serine)-157 and [Serine](/source/Serine)-161 has been shown to be essential for normal [T-tubule](/source/T-tubule) organization and intracellular [calcium](/source/calcium) transient kinetics.[{{cite journal | vauthors = Tian LF, Li HY, Jin BF, Pan X, Man JH, Zhang PJ, Li WH, Liang B, Liu H, Zhao J, Gong WL, Zhou T, Zhang XM | title = MDM2 interacts with and downregulates a sarcomeric protein, TCAP | journal = Biochemical and Biophysical Research Communications | volume = 345 | issue = 1 | pages = 355–61 | date = Jun 2006 | pmid = 16678796 | doi = 10.1016/j.bbrc.2006.04.108 }}.] Telethonin specifically interacts with the pro-apoptotic protein [Siva](/source/SIVA1), suggesting that Telethonin may be involved in the mechanism underlying [Coxsackievirus](/source/Coxsackievirus) B3 infection in acute and chronic [myocarditis](/source/myocarditis)[{{cite journal | vauthors = Mihatsch K, Nestler M, Saluz HP, Henke A, Munder T | title = Proapoptotic protein Siva binds to the muscle protein telethonin in cardiomyocytes during coxsackieviral infection | journal = Cardiovascular Research | volume = 81 | issue = 1 | pages = 108–15 | date = Jan 2009 | pmid = 18849585 | doi = 10.1093/cvr/cvn276 | doi-access = free }}]
Telethonin was also identified to be targeted and regulated by transcriptional activators [CLOCK](/source/CLOCK) and [BMAL1](/source/BMAL1), thus demonstrating that ''TCAP'' is a circadian regulated gene.[{{cite journal | vauthors = Podobed PS, Alibhai FJ, Chow CW, Martino TA | title = Circadian regulation of myocardial sarcomeric Titin-cap (Tcap, telethonin): identification of cardiac clock-controlled genes using open access bioinformatics data | journal = PLOS ONE | volume = 9 | issue = 8 | article-number = e104907 | date = 2014 | pmid = 25121604 | doi = 10.1371/journal.pone.0104907 | pmc=4133362| bibcode = 2014PLoSO...9j4907P | doi-access = free }}]
==Clinical Significance==
Mutations in this gene are associated with [limb-girdle muscular dystrophy](/source/limb-girdle_muscular_dystrophy) type R7 (previously 2G),[{{cite journal | vauthors = Vainzof M, Moreira ES, Suzuki OT, Faulkner G, Valle G, Beggs AH, Carpen O, Ribeiro AF, Zanoteli E, Gurgel-Gianneti J, Tsanaclis AM, Silva HC, Passos-Bueno MR, Zatz M | title = Telethonin protein expression in neuromuscular disorders | journal = Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease | volume = 1588 | issue = 1 | pages = 33–40 | date = Oct 2002 | pmid = 12379311 | doi=10.1016/s0925-4439(02)00113-8| url = http://repositorio.unifesp.br/handle/11600/27000 | doi-access = free | url-access = subscription }}] [hypertrophic cardiomyopathy](/source/hypertrophic_cardiomyopathy),[{{cite journal | vauthors = Andersen PS, Havndrup O, Hougs L, Sørensen KM, Jensen M, Larsen LA, Hedley P, Thomsen AR, Moolman-Smook J, Christiansen M, Bundgaard H | title = Diagnostic yield, interpretation, and clinical utility of mutation screening of sarcomere encoding genes in Danish hypertrophic cardiomyopathy patients and relatives | journal = Human Mutation | volume = 30 | issue = 3 | pages = 363–70 | date = Mar 2009 | pmid = 19035361 | doi = 10.1002/humu.20862 | s2cid = 30898294 }}][{{cite journal | vauthors = Bos JM, Poley RN, Ny M, Tester DJ, Xu X, Vatta M, Towbin JA, Gersh BJ, Ommen SR, Ackerman MJ | title = Genotype-phenotype relationships involving hypertrophic cardiomyopathy-associated mutations in titin, muscle LIM protein, and telethonin | journal = Molecular Genetics and Metabolism | volume = 88 | issue = 1 | pages = 78–85 | date = May 2006 | pmid = 16352453 | doi = 10.1016/j.ymgme.2005.10.008 | pmc=2756511}}][{{cite journal | vauthors = Theis JL, Bos JM, Bartleson VB, Will ML, Binder J, Vatta M, Towbin JA, Gersh BJ, Ommen SR, Ackerman MJ | title = Echocardiographic-determined septal morphology in Z-disc hypertrophic cardiomyopathy | journal = Biochemical and Biophysical Research Communications | volume = 351 | issue = 4 | pages = 896–902 | date = Dec 2006 | pmid = 17097056 | doi = 10.1016/j.bbrc.2006.10.119 }}] [dilated cardiomyopathy](/source/dilated_cardiomyopathy),[{{cite journal | vauthors = Rampersaud E, Siegfried JD, Norton N, Li D, Martin E, Hershberger RE | title = Rare variant mutations identified in pediatric patients with dilated cardiomyopathy | journal = Progress in Pediatric Cardiology | volume = 31 | issue = 1 | pages = 39–47 | date = Jan 2011 | pmid = 21483645 | doi = 10.1016/j.ppedcard.2010.11.008 | pmc=3072577}}][{{cite journal | vauthors = Hirtle-Lewis M, Desbiens K, Ruel I, Rudzicz N, Genest J, Engert JC, Giannetti N | title = The genetics of dilated cardiomyopathy: a prioritized candidate gene study of LMNA, TNNT2, TCAP, and PLN | journal = Clinical Cardiology | volume = 36 | issue = 10 | pages = 628–33 | date = Oct 2013 | pmid = 24037902 | doi = 10.1002/clc.22193 | pmc = 6649360 }}] idiopathic [cardiomyopathy](/source/cardiomyopathy),[{{cite journal | vauthors = Hershberger RE, Parks SB, Kushner JD, Li D, Ludwigsen S, Jakobs P, Nauman D, Burgess D, Partain J, Litt M | title = Coding sequence mutations identified in MYH7, TNNT2, SCN5A, CSRP3, LBD3, and TCAP from 313 patients with familial or idiopathic dilated cardiomyopathy | journal = Clinical and Translational Science | volume = 1 | issue = 1 | pages = 21–6 | date = May 2008 | pmid = 19412328 | doi = 10.1111/j.1752-8062.2008.00017.x | pmc=2633921}}] and gastrointestinal smooth muscle-related diseases.[{{cite journal | vauthors = Hayashi T, Arimura T, Itoh-Satoh M, Ueda K, Hohda S, Inagaki N, Takahashi M, Hori H, Yasunami M, Nishi H, Koga Y, Nakamura H, Matsuzaki M, Choi BY, Bae SW, You CW, Han KH, Park JE, Knöll R, Hoshijima M, Chien KR, Kimura A | title = Tcap gene mutations in hypertrophic cardiomyopathy and dilated cardiomyopathy | journal = Journal of the American College of Cardiology | volume = 44 | issue = 11 | pages = 2192–201 | date = Dec 2004 | pmid = 15582318 | doi = 10.1016/j.jacc.2004.08.058 | doi-access = free }}] Mutations in [Titin](/source/Titin) associated with [dilated cardiomyopathy](/source/dilated_cardiomyopathy), including [Val](/source/Valine)54[Met](/source/Methionine), have been shown specifically to impair binding of [Titin](/source/Titin) with Telethonin.[{{cite journal | vauthors = Itoh-Satoh M, Hayashi T, Nishi H, Koga Y, Arimura T, Koyanagi T, Takahashi M, Hohda S, Ueda K, Nouchi T, Hiroe M, Marumo F, Imaizumi T, Yasunami M, Kimura A | s2cid = 1824336 | title = Titin mutations as the molecular basis for dilated cardiomyopathy | journal = Biochemical and Biophysical Research Communications | volume = 291 | issue = 2 | pages = 385–93 | date = Feb 2002 | pmid = 11846417 | doi = 10.1006/bbrc.2002.6448 }}] In a mouse model of [dilated cardiomyopathy](/source/dilated_cardiomyopathy), recapitulating the human [dilated cardiomyopathy](/source/dilated_cardiomyopathy) mutation in [MLP](/source/CSRP3), [Trp](/source/Tryptophan)4[Arg](/source/Arginine), studies have found that this mutation disrupts normal binding and localization of [MLP](/source/CSRP3) with Telethonin.[{{cite journal | vauthors = Nakano N, Hori H, Abe M, Shibata H, Arimura T, Sasaoka T, Sawabe M, Chida K, Arai T, Nakahara K, Kubo T, Sugimoto K, Katsuya T, Ogihara T, Doi Y, Izumi T, Kimura A | title = Interaction of BMP10 with Tcap may modulate the course of hypertensive cardiac hypertrophy | journal = American Journal of Physiology. Heart and Circulatory Physiology | volume = 293 | issue = 6 | pages = H3396–403 | date = Dec 2007 | pmid = 17921333 | doi = 10.1152/ajpheart.00311.2007 | s2cid = 45084505 }}]
== Interactions ==
Telethonin has been shown to [interact](/source/Protein-protein_interaction) with:
{{div col|colwidth=20em}}
* [ANKRD2](/source/ANKRD2),[{{cite journal | vauthors = Kojic S, Medeot E, Guccione E, Krmac H, Zara I, Martinelli V, Valle G, Faulkner G | title = The Ankrd2 protein, a link between the sarcomere and the nucleus in skeletal muscle | journal = Journal of Molecular Biology | volume = 339 | issue = 2 | pages = 313–25 | date = May 2004 | pmid = 15136035 | doi = 10.1016/j.jmb.2004.03.071 }}]
* [BMP10](/source/BMP10),[{{cite journal | vauthors = Nakano N, Hori H, Abe M, Shibata H, Arimura T, Sasaoka T, Sawabe M, Chida K, Arai T, Nakahara K, Kubo T, Sugimoto K, Katsuya T, Ogihara T, Doi Y, Izumi T, Kimura A | title = Interaction of BMP10 with Tcap may modulate the course of hypertensive cardiac hypertrophy | journal = American Journal of Physiology. Heart and Circulatory Physiology | volume = 293 | issue = 6 | pages = H3396–403 | date = Dec 2007 | pmid = 17921333 | doi = 10.1152/ajpheart.00311.2007 | s2cid = 45084505 }}]
* [MLP](/source/CSRP3),[{{cite journal | vauthors = Tian LF, Li HY, Jin BF, Pan X, Man JH, Zhang PJ, Li WH, Liang B, Liu H, Zhao J, Gong WL, Zhou T, Zhang XM | title = MDM2 interacts with and downregulates a sarcomeric protein, TCAP | journal = Biochemical and Biophysical Research Communications | volume = 345 | issue = 1 | pages = 355–61 | date = Jun 2006 | pmid = 16678796 | doi = 10.1016/j.bbrc.2006.04.108 }}]
* [MSTN](/source/MSTN),[{{cite journal | vauthors = Nicholas G, Thomas M, Langley B, Somers W, Patel K, Kemp CF, Sharma M, Kambadur R | title = Titin-cap associates with, and regulates secretion of, Myostatin | journal = Journal of Cellular Physiology | volume = 193 | issue = 1 | pages = 120–31 | date = Oct 2002 | pmid = 12209887 | doi = 10.1002/jcp.10158 | s2cid = 8866409 | url = http://researchonline.rvc.ac.uk/id/eprint/1181/ | url-access = subscription }}]
* [MYOZ1](/source/MYOZ1),[{{cite journal | vauthors = Frey N, Olson EN | title = Calsarcin-3, a novel skeletal muscle-specific member of the calsarcin family, interacts with multiple Z-disc proteins | journal = The Journal of Biological Chemistry | volume = 277 | issue = 16 | pages = 13998–4004 | date = Apr 2002 | pmid = 11842093 | doi = 10.1074/jbc.M200712200 | doi-access = free }}][{{cite journal | vauthors = Faulkner G, Pallavicini A, Comelli A, Salamon M, Bortoletto G, Ievolella C, Trevisan S, Kojic' S, Dalla Vecchia F, Laveder P, Valle G, Lanfranchi G | title = FATZ, a filamin-, actinin-, and telethonin-binding protein of the Z-disc of skeletal muscle | journal = The Journal of Biological Chemistry | volume = 275 | issue = 52 | pages = 41234–42 | date = Dec 2000 | pmid = 10984498 | doi = 10.1074/jbc.M007493200 | doi-access = free | hdl = 11368/1698365 | hdl-access = free }}]
* [MYOZ2](/source/MYOZ2),[{{cite journal | vauthors = Haworth RS, Cuello F, Herron TJ, Franzen G, Kentish JC, Gautel M, Avkiran M | title = Protein kinase D is a novel mediator of cardiac troponin I phosphorylation and regulates myofilament function | journal = Circulation Research | volume = 95 | issue = 11 | pages = 1091–9 | date = Nov 2004 | pmid = 15514163 | doi = 10.1161/01.RES.0000149299.34793.3c | doi-access = free }}]
* [SCN5A](/source/SCN5A),[{{cite journal | vauthors = Mihatsch K, Nestler M, Saluz HP, Henke A, Munder T | title = Proapoptotic protein Siva binds to the muscle protein telethonin in cardiomyocytes during coxsackieviral infection | journal = Cardiovascular Research | volume = 81 | issue = 1 | pages = 108–15 | date = Jan 2009 | pmid = 18849585 | doi = 10.1093/cvr/cvn276 | doi-access = free }}] and
* [Titin](/source/Titin),[{{cite journal | vauthors = Mayans O, van der Ven PF, Wilm M, Mues A, Young P, Fürst DO, Wilmanns M, Gautel M | title = Structural basis for activation of the titin kinase domain during myofibrillogenesis | journal = Nature | volume = 395 | issue = 6705 | pages = 863–9 | date = Oct 1998 | pmid = 9804419 | doi = 10.1038/27603 | bibcode = 1998Natur.395..863M | s2cid = 4426977 }}][{{cite journal | vauthors = Zou P, Gautel M, Geerlof A, Wilmanns M, Koch MH, Svergun DI | title = Solution scattering suggests cross-linking function of telethonin in the complex with titin | journal = The Journal of Biological Chemistry | volume = 278 | issue = 4 | pages = 2636–44 | date = Jan 2003 | pmid = 12446666 | doi = 10.1074/jbc.M210217200 | doi-access = free }}][{{cite journal | vauthors = Mues A, van der Ven PF, Young P, Fürst DO, Gautel M | title = Two immunoglobulin-like domains of the Z-disc portion of titin interact in a conformation-dependent way with telethonin | journal = FEBS Letters | volume = 428 | issue = 1–2 | pages = 111–4 | date = May 1998 | pmid = 9645487 | doi = 10.1016/s0014-5793(98)00501-8| s2cid = 11786578 | doi-access = free }}]
{{Div col end}}
== References ==
{{reflist|33em}}
== Further reading ==
{{refbegin|33em}}
* {{cite journal | vauthors = Faulkner G, Lanfranchi G, Valle G | title = Telethonin and other new proteins of the Z-disc of skeletal muscle | journal = IUBMB Life | volume = 51 | issue = 5 | pages = 275–82 | date = May 2001 | pmid = 11699871 | doi = 10.1080/152165401317190761 | s2cid = 23688131 | doi-access = free }}
* {{cite journal | vauthors = Moreira ES, Vainzof M, Marie SK, Sertié AL, Zatz M, Passos-Bueno MR | title = The seventh form of autosomal recessive limb-girdle muscular dystrophy is mapped to 17q11-12 | journal = American Journal of Human Genetics | volume = 61 | issue = 1 | pages = 151–9 | date = Jul 1997 | pmid = 9245996 | pmc = 1715843 | doi = 10.1086/513889 }}
* {{cite journal | vauthors = Mues A, van der Ven PF, Young P, Fürst DO, Gautel M | title = Two immunoglobulin-like domains of the Z-disc portion of titin interact in a conformation-dependent way with telethonin | journal = FEBS Letters | volume = 428 | issue = 1–2 | pages = 111–4 | date = May 1998 | pmid = 9645487 | doi = 10.1016/S0014-5793(98)00501-8 | s2cid = 11786578 | doi-access = free }}
* {{cite journal | vauthors = Mayans O, van der Ven PF, Wilm M, Mues A, Young P, Fürst DO, Wilmanns M, Gautel M | title = Structural basis for activation of the titin kinase domain during myofibrillogenesis | journal = Nature | volume = 395 | issue = 6705 | pages = 863–9 | date = Oct 1998 | pmid = 9804419 | doi = 10.1038/27603 | bibcode = 1998Natur.395..863M | s2cid = 4426977 }}
* {{cite journal | vauthors = Moreira ES, Wiltshire TJ, Faulkner G, Nilforoushan A, Vainzof M, Suzuki OT, Valle G, Reeves R, Zatz M, Passos-Bueno MR, Jenne DE | title = Limb-girdle muscular dystrophy type 2G is caused by mutations in the gene encoding the sarcomeric protein telethonin | journal = Nature Genetics | volume = 24 | issue = 2 | pages = 163–6 | date = Feb 2000 | pmid = 10655062 | doi = 10.1038/72822 | s2cid = 8698402 }}
* {{cite journal | vauthors = Faulkner G, Pallavicini A, Comelli A, Salamon M, Bortoletto G, Ievolella C, Trevisan S, Kojic' S, Dalla Vecchia F, Laveder P, Valle G, Lanfranchi G | title = FATZ, a filamin-, actinin-, and telethonin-binding protein of the Z-disc of skeletal muscle | journal = The Journal of Biological Chemistry | volume = 275 | issue = 52 | pages = 41234–42 | date = Dec 2000 | pmid = 10984498 | doi = 10.1074/jbc.M007493200 | doi-access = free | hdl = 11368/1698365 | hdl-access = free }}
* {{cite journal | vauthors = Schröder R, Reimann J, Iakovenko A, Mues A, Bönnemann CG, Matten J, Gautel M | title = Early and selective disappearance of telethonin protein from the sarcomere in neurogenic atrophy | journal = Journal of Muscle Research and Cell Motility | volume = 22 | issue = 3 | pages = 259–64 | year = 2002 | pmid = 11763198 | doi = 10.1023/A:1012242011109 | s2cid = 22553971 }}
* {{cite journal | vauthors = Frey N, Olson EN | title = Calsarcin-3, a novel skeletal muscle-specific member of the calsarcin family, interacts with multiple Z-disc proteins | journal = The Journal of Biological Chemistry | volume = 277 | issue = 16 | pages = 13998–4004 | date = Apr 2002 | pmid = 11842093 | doi = 10.1074/jbc.M200712200 | doi-access = free }}
* {{cite journal | vauthors = Nicholas G, Thomas M, Langley B, Somers W, Patel K, Kemp CF, Sharma M, Kambadur R | title = Titin-cap associates with, and regulates secretion of, Myostatin | journal = Journal of Cellular Physiology | volume = 193 | issue = 1 | pages = 120–31 | date = Oct 2002 | pmid = 12209887 | doi = 10.1002/jcp.10158 | s2cid = 8866409 | url = http://researchonline.rvc.ac.uk/id/eprint/1181/ | url-access = subscription }}
* {{cite journal | vauthors = Zou P, Gautel M, Geerlof A, Wilmanns M, Koch MH, Svergun DI | title = Solution scattering suggests cross-linking function of telethonin in the complex with titin | journal = The Journal of Biological Chemistry | volume = 278 | issue = 4 | pages = 2636–44 | date = Jan 2003 | pmid = 12446666 | doi = 10.1074/jbc.M210217200 | doi-access = free }}
* {{cite journal | vauthors = Knöll R, Hoshijima M, Hoffman HM, Person V, Lorenzen-Schmidt I, Bang ML, Hayashi T, Shiga N, Yasukawa H, Schaper W, McKenna W, Yokoyama M, Schork NJ, Omens JH, McCulloch AD, Kimura A, Gregorio CC, Poller W, Schaper J, Schultheiss HP, Chien KR | title = The cardiac mechanical stretch sensor machinery involves a Z disc complex that is defective in a subset of human dilated cardiomyopathy | journal = Cell | volume = 111 | issue = 7 | pages = 943–55 | date = Dec 2002 | pmid = 12507422 | doi = 10.1016/S0092-8674(02)01226-6 | s2cid = 15082967 | doi-access = free }}
* {{cite journal | vauthors = Katoh M, Katoh M | title = Evolutionary recombination hotspot around GSDML-GSDM locus is closely linked to the oncogenomic recombination hotspot around the PPP1R1B-ERBB2-GRB7 amplicon | journal = International Journal of Oncology | volume = 24 | issue = 4 | pages = 757–63 | date = Apr 2004 | pmid = 15010812 | doi = 10.3892/ijo.24.4.757 }}
* {{cite journal | vauthors = Kojic S, Medeot E, Guccione E, Krmac H, Zara I, Martinelli V, Valle G, Faulkner G | title = The Ankrd2 protein, a link between the sarcomere and the nucleus in skeletal muscle | journal = Journal of Molecular Biology | volume = 339 | issue = 2 | pages = 313–25 | date = May 2004 | pmid = 15136035 | doi = 10.1016/j.jmb.2004.03.071 }}
* {{cite journal | vauthors = Hayashi T, Arimura T, Itoh-Satoh M, Ueda K, Hohda S, Inagaki N, Takahashi M, Hori H, Yasunami M, Nishi H, Koga Y, Nakamura H, Matsuzaki M, Choi BY, Bae SW, You CW, Han KH, Park JE, Knöll R, Hoshijima M, Chien KR, Kimura A | title = Tcap gene mutations in hypertrophic cardiomyopathy and dilated cardiomyopathy | journal = Journal of the American College of Cardiology | volume = 44 | issue = 11 | pages = 2192–201 | date = Dec 2004 | pmid = 15582318 | doi = 10.1016/j.jacc.2004.08.058 | doi-access = free }}
{{refend}}
== External links ==
* [https://www.ncbi.nlm.nih.gov/books/NBK1768/ GeneReviews/NIH/NCBI/UW entry on Familial Hypertrophic Cardiomyopathy Overview]
{{PDB Gallery|geneid=8557}}
{{Muscle tissue}}
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Adapted from the Wikipedia article [Telethonin](https://en.wikipedia.org/wiki/Telethonin) by Wikipedia contributors ([contributor history](https://en.wikipedia.org/wiki/Telethonin?action=history)). Available under [Creative Commons Attribution-ShareAlike 4.0 International](https://creativecommons.org/licenses/by-sa/4.0/). Changes may have been made.