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<h1 id="firstHeading" class="firstHeading mw-first-heading"><span class="mw-page-title-main">Perforin</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_1.C.39.2.1" style="font-size:90%; margin-top:0; width:350px;" summary="Infobox Protein">
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<th colspan="3" style="background:#90EE90; color:#202122;">Perforin-1
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<p>Vorhandene Strukturdaten: <span class=""><a rel="nofollow" class="external text" href="https://www.rcsb.org/structure/2qp2">2qp2</a></span>
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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;">534 Aminosäuren
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<td><a href="Sekund%C3%A4rstruktur" title="Sekundärstruktur">Sekundär-</a> bis <a href="Quart%C3%A4rstruktur" title="Quartärstruktur">Quartärstruktur</a>
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<td colspan="2" style="text-align:center;">multipass Membranprotein
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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-Namen</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=9360">PRF1</a></i>, FLH2, HPLH2, MGC65093, P1, PFP
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<td>Externe IDs
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<td colspan="2" class="">
<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/170280">170280</a></li>
<li><a href="UniProt" title="UniProt">UniProt</a> <a rel="nofollow" class="external text" href="https://www.uniprot.org/uniprotkb/P14222">P14222</a></li>
<li><a href="Mouse_Genome_Informatics" title="Mouse Genome Informatics">MGI</a>: <a rel="nofollow" class="external text" href="https://www.informatics.jax.org/marker/MGI:97551">97551</a></li></ul>
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<th colspan="3" style="background:#90EE90; color:#202122;">Transporter-Klassifikation
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<td><a href="Transporter_Classification_Database" title="Transporter Classification Database">TCDB</a>
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<td colspan="2" class="" style="text-align:center;"><a rel="nofollow" class="external text" href="https://tcdb.org/search/result.php?tc=1.C.39.2.1">1.C.39.2.1</a>
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<td><a href="Membrantransport" title="Membrantransport">Bezeichnung</a>
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<td colspan="2" style="text-align:center;">Komplementprotein C9-Familie
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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=P14222">Hovergen</a>
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<td style="background:#C3FDB8; color:#202122;">Übergeordnetes <a href="Taxon" title="Taxon">Taxon</a>
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<td colspan="2" style="text-align:center;"><a href="Euteleostomi" title="Euteleostomi">Euteleostomi</a>
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</tbody></table><p><span class="editoronly" style="display:none;"></span>
</p><p><b>Perforin</b> (<b>PRF1</b>) ist ein zytolytisches <a href="Protein" title="Protein">Protein</a>, welches in <a href="Granula" title="Granula">Granula</a> der <a href="Cytotoxische_T-Zelle" class="mw-redirect" title="Cytotoxische T-Zelle">cytotoxischen T-Zellen</a> (CD8 positiven <a href="T-Lymphozyt" title="T-Lymphozyt">T-Lymphozyten</a>) und <a href="NK-Zelle" title="NK-Zelle">NK-Zellen</a> gefunden werden kann. Bei der <a href="Degranulation" class="mw-redirect" title="Degranulation">Degranulation</a> perforiert es die <a href="Zellmembran" title="Zellmembran">Zellmembran</a> der Zielzelle und formt eine Pore. Durch diese Pore dringen <a href="Granzyme" title="Granzyme">Granzyme</a> B in die Zielzelle ein und lösen eine <a href="Apoptose" title="Apoptose">Apoptose</a> aus. Bei der Immuntherapie wird die Generierung von Perforin zur Tumorbekämpfung genutzt.
</p><p><a href="Mutation" title="Mutation">Mutationen</a> im <i>PRF1</i>-<a href="Gen" title="Gen">Gen</a> können <a href="H%C3%A4mophagozytische_Lymphohistiozytose" title="Hämophagozytische Lymphohistiozytose">hämophagozytische Lymphohistiozytose</a> Typ 2 (FHL2) verursachen.<sup id="cite_ref-1" class="reference"><a href="#cite_note-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-2" class="reference"><a href="#cite_note-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup>
</p>
<div class="mw-heading mw-heading2"><h2 id="Literatur">Literatur</h2></div>
<ul><li>Pipkin ME, Lieberman J: <cite class="lang" lang="en" dir="auto" style="font-style:italic">Delivering the kiss of death: progress on understanding how perforin works</cite>. In: <cite class="lang" lang="en" dir="auto" style="font-style:italic">Curr. Opin. Immunol.</cite> 19. Jahrgang, <span style="white-space:nowrap">Nr.<span style="display:inline-block;width:.2em"> </span>3</span>, Juni 2007, <span style="white-space:nowrap">S.<span style="display:inline-block;width:.2em"> </span>301–8</span>, <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/10.1016/j.coi.2007.04.011">10.1016/j.coi.2007.04.011</a></span>, <a class="external mw-magiclink-pmid" rel="nofollow" href="https://www.ncbi.nlm.nih.gov/pubmed/17433871?dopt=Abstract">PMID 17433871</a> (englisch).<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rfr_id=info:sid/de.wikipedia.org:Perforin&rft.atitle=Delivering+the+kiss+of+death%3A+progress+on+understanding+how+perforin+works&rft.au=Pipkin+ME%2C+Lieberman+J&rft.date=2007-06&rft.doi=10.1016%2Fj.coi.2007.04.011&rft.genre=journal&rft.issue=3&rft.jtitle=Curr.+Opin.+Immunol.&rft.pages=301-8&rft.pmid=17433871&rft.volume=19.+Jahrgang" style="display:none"> </span></li>
<li>Kurschus FC, Fellows E, Stegmann E, Jenne DE: <cite class="lang" lang="en" dir="auto" style="font-style:italic">Granzyme B delivery via perforin is restricted by size, but not by heparan sulfate-dependent endocytosis</cite>. In: <cite class="lang" lang="en" dir="auto" style="font-style:italic">Proc. Natl. Acad. Sci. U.S.A.</cite> 105. Jahrgang, <span style="white-space:nowrap">Nr.<span style="display:inline-block;width:.2em"> </span>37</span>, September 2008, <span style="white-space:nowrap">S.<span style="display:inline-block;width:.2em"> </span>13799–804</span>, <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/10.1073/pnas.0801724105">10.1073/pnas.0801724105</a></span>, <a class="external mw-magiclink-pmid" rel="nofollow" href="https://www.ncbi.nlm.nih.gov/pubmed/18772390?dopt=Abstract">PMID 18772390</a>, <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2544534/">PMC 2544534</a> (freier Volltext) – (englisch).<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rfr_id=info:sid/de.wikipedia.org:Perforin&rft.atitle=Granzyme+B+delivery+via+perforin+is+restricted+by+size%2C+but+not+by+heparan+sulfate-dependent+endocytosis&rft.au=Kurschus+FC%2C+Fellows+E%2C+Stegmann+E%2C+...&rft.date=2008-09&rft.doi=10.1073%2Fpnas.0801724105&rft.genre=journal&rft.issue=37&rft.jtitle=Proc.+Natl.+Acad.+Sci.+U.S.A.&rft.pages=13799-804&rft.pmc=2544534&rft.pmid=18772390&rft.volume=105.+Jahrgang" style="display:none"> </span></li>
<li>Bolitho P, Voskoboinik I, Trapani JA, <a href="Mark_J._Smyth" title="Mark J. Smyth">Smyth MJ</a>: <cite class="lang" lang="en" dir="auto" style="font-style:italic">Apoptosis induced by the lymphocyte effector molecule perforin</cite>. In: <cite class="lang" lang="en" dir="auto" style="font-style:italic">Curr. Opin. Immunol.</cite> 19. Jahrgang, <span style="white-space:nowrap">Nr.<span style="display:inline-block;width:.2em"> </span>3</span>, Juni 2007, <span style="white-space:nowrap">S.<span style="display:inline-block;width:.2em"> </span>339–47</span>, <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/10.1016/j.coi.2007.04.007">10.1016/j.coi.2007.04.007</a></span>, <a class="external mw-magiclink-pmid" rel="nofollow" href="https://www.ncbi.nlm.nih.gov/pubmed/17442557?dopt=Abstract">PMID 17442557</a> (englisch).<span class="Z3988" title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rfr_id=info:sid/de.wikipedia.org:Perforin&rft.atitle=Apoptosis+induced+by+the+lymphocyte+effector+molecule+perforin&rft.au=Bolitho+P%2C+Voskoboinik+I%2C+Trapani+JA%2C+...&rft.date=2007-06&rft.doi=10.1016%2Fj.coi.2007.04.007&rft.genre=journal&rft.issue=3&rft.jtitle=Curr.+Opin.+Immunol.&rft.pages=339-47&rft.pmid=17442557&rft.volume=19.+Jahrgang" style="display:none"> </span></li></ul>
<div class="mw-heading mw-heading2"><h2 id="Weblinks">Weblinks</h2></div>
<ul><li>Eintrag zu <a rel="nofollow" class="external text" href="https://www.orpha.net/de/disease/detail/540"><i>Lymphohistiozytose, hämophagozytische, familiäre Form.</i></a> In: <i><a href="Orphanet" title="Orphanet">Orphanet</a></i> (Datenbank für seltene Krankheiten)<span class="editoronly" style="display:none;"></span></li></ul>
<div class="mw-heading mw-heading2"><h2 id="Einzelnachweise">Einzelnachweise</h2></div>
<ol class="references">
<li id="cite_note-1"><span class="mw-cite-backlink"><a href="#cite_ref-1">↑</a></span> <span class="reference-text"><a href="UniProt" title="UniProt">UniProt</a> <a rel="nofollow" class="external text" href="https://www.uniprot.org/uniprotkb/P14222">P14222</a></span>
</li>
<li id="cite_note-2"><span class="mw-cite-backlink"><a href="#cite_ref-2">↑</a></span> <span class="reference-text"><a rel="nofollow" class="external text" href="https://omim.org/entry/603553"><i>Perforin.</i></a> In: <i><span lang="en"><a href="Online_Mendelian_Inheritance_in_Man" title="Online Mendelian Inheritance in Man">Online Mendelian Inheritance in Man</a></span>.</i> (englisch)<span class="editoronly" style="display:none;"></span></span>
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