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<h1 id="firstHeading" class="firstHeading mw-first-heading"><span class="mw-page-title-main">Telethonin</span></h1>
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<div id="mw-content-text" class="mw-body-content mw-content-ltr" lang="de" dir="ltr"><div class="mw-content-ltr mw-parser-output" lang="de" dir="ltr"><table class="wikitable hintergrundfarbe-basis infobox float-right" id="Vorlage_Infobox_Protein_Telethonin" style="font-size:90%; margin-top:0; width:350px;" summary="Infobox Protein">
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<th colspan="3" style="background:#90EE90; color:#202122;">Telethonin
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<td colspan="3" class="hintergrundfarbe1" style="text-align:center; font-size:smaller; font-weight:bold;">nach <a href="Protein_Data_Bank" title="Protein Data Bank">PDB</a> <a rel="nofollow" class="external text" href="https://www.rcsb.org/structure/1ya5">1ya5</a>
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<th colspan="3" style="background:#90EE90; color:#202122;;">Eigenschaften des menschlichen Proteins
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<td><a href="Molare_Masse" title="Molare Masse">Masse</a>/Länge <a href="Prim%C3%A4rstruktur" title="Primärstruktur">Primärstruktur</a>
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<td colspan="2" style="text-align:center;">167 AA, 19 kDa
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<th colspan="3" style="background:#90EE90; color:#202122;">Bezeichner
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<td><a href="Human_Genome_Organisation" title="Human Genome Organisation">Gen-Name</a>
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<td colspan="2" class="" style="text-align:center;"><i><a rel="nofollow" class="external text" href="https://www.genenames.org/tools/search/#!/all?query=11610">TCAP</a></i>
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<td>Externe IDs
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<ul><li><a href="Online_Mendelian_Inheritance_in_Man" title="Online Mendelian Inheritance in Man">OMIM</a>: <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/omim/604488">604488</a></li>
<li><a href="UniProt" title="UniProt">UniProt</a> <a rel="nofollow" class="external text" href="https://www.uniprot.org/uniprotkb/O15273">O15273</a></li></ul>
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<th colspan="3" style="background:#90EE90; color:#202122;">Vorkommen
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<td style="background:#C3FDB8; color:#202122;">Homologie-Familie
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<td colspan="2" style="text-align:center;"><a rel="nofollow" class="external text" href="http://hogenom.univ-lyon1.fr/query_sequence?seq=O15273">Hovergen</a>
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</p><p><b>Telethonin</b>, auch <b>Titin cap protein</b>, ist ein überwiegend in Muskel- und Herzmuskelzellen von <a href="Eukaryoten" title="Eukaryoten">Eukaryoten</a> vorkommendes <a href="Protein" title="Protein">Protein</a>. Beim Menschen wird es kodiert vom <i>TCAP</i>-Gen und ist auf dem langen Arm von <a href="Chromosom_17_(Mensch)" title="Chromosom 17 (Mensch)">Chromosom 17</a> (17q12) lokalisiert.
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<div class="mw-heading mw-heading2"><h2 id="Funktion">Funktion</h2></div>
<p>Telethonin ist ein Strukturprotein der <a href="Z-Scheibe" class="mw-redirect" title="Z-Scheibe">Z-Scheibe</a> der <a href="Sarkomer" title="Sarkomer">Sarkomere</a> von Muskelzellen. Es besitzt jeweils zwei Bindestellen für <a href="Titin" title="Titin">Titin</a>, ein Protein, das zwischen beiden Z-Scheiben verankert wird. Darüber hinaus interagiert es mit
Myozenin-1, Myozenin-2, Myozenin-3, Cysteine and glycine-rich protein 3 und Ankyrin repeat domain-containing protein 2.
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<div class="mw-heading mw-heading2"><h2 id="Medizinische_Bedeutung">Medizinische Bedeutung</h2></div>
<p>Mutationen im <i>TCAP</i>-Gen konnten bei der <a href="Gliederg%C3%BCrteldystrophie_2G" title="Gliedergürteldystrophie 2G">Gliedergürteldystrophie 2G</a> (LGMD2G), der familiären hypertrophen Kardiomyopathie und einer Form der <a href="Dilatative_Kardiomyopathie" title="Dilatative Kardiomyopathie">dilatativen Kardiomyopathie</a> (CMD1N) nachgewiesen werden.
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<div class="mw-heading mw-heading2"><h2 id="Literatur">Literatur</h2></div>
<ul><li>P. Zou, N. Pinotsis u. a.: <i>Palindromic assembly of the giant muscle protein titin in the sarcomeric Z-disk.</i> In: <i>Nature.</i> Band 439, Nummer 7073, Januar 2006, S. 229–233, <a href="Digital_Object_Identifier" title="Digital Object Identifier">doi</a>:<span class="uri-handle" style="white-space:nowrap"><a rel="nofollow" class="external text" href="https://doi.org/10.1038/nature04343">10.1038/nature04343</a></span>. <a class="external mw-magiclink-pmid" rel="nofollow" href="https://www.ncbi.nlm.nih.gov/pubmed/16407954?dopt=Abstract">PMID 16407954</a>.</li>
<li>G. Valle, G. Faulkner u. a.: <i>Telethonin, a novel sarcomeric protein of heart and skeletal muscle.</i> In: <i>FEBS letters.</i> Band 415, Nummer 2, September 1997, S. 163–168, <a class="external mw-magiclink-pmid" rel="nofollow" href="https://www.ncbi.nlm.nih.gov/pubmed/9350988?dopt=Abstract">PMID 9350988</a>.</li></ul></div><!--htdig_noindex--><div><div class="zim-footer">
Dieser Artikel wurde von <a class="external text" title="Zuletzt bearbeitet am 2017-02-12" href="https://de.wikipedia.org/wiki/?title=Telethonin&oldid=162571791">Wikipedia</a> herausgegeben. Der Text ist unter <a class="external text" href="https://creativecommons.org/licenses/by-sa/4.0/deed.de">Creative Commons Attribution-Share Alike 4.0</a> verfügbar, sofern nicht anders angegeben. Für die Mediendateien können zusätzliche Bedingungen gelten.
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