Micron Document
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<h1 id="firstHeading" class="firstHeading mw-first-heading"><span class="mw-page-title-main">Proconvertin</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_Faktor_VIIa" style="font-size:90%; margin-top:0; width:350px;" summary="Infobox Protein">

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<th colspan="3" style="background:#90EE90; color:#202122;">Faktor VIIa
</th></tr>
<tr style="text-align:center;">
<td colspan="3"><span typeof="mw:File"></span>
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<td colspan="3" class="hintergrundfarbe1" style="text-align:center; font-size:smaller; font-weight:bold;">Thromboplastin (blau) komplexiert mit Faktor VIIa nach <a href="Protein_Data_Bank" title="Protein Data Bank">PDB</a>&nbsp;<a rel="nofollow" class="external text" href="https://www.rcsb.org/structure/1DAN">1DAN</a>
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<tr>
<td colspan="3" class="hintergrundfarbe1" style="font-size:smaller;">
<p>Vorhandene Strukturdaten: s. UniProt
</p>
</td></tr>
<tr>
<th colspan="3" style="background:#90EE90; color:#202122;;">Eigenschaften des menschlichen Proteins
</th></tr>
<tr>
<td><a href="Molare_Masse" title="Molare Masse">Masse</a>/Länge <a href="Prim%C3%A4rstruktur" title="Primärstruktur">Primärstruktur</a>
</td>
<td colspan="2" style="text-align:center;">406 = 152+254 Aminosäuren
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<tr>
<td><a href="Sekund%C3%A4rstruktur" title="Sekundärstruktur">Sekundär-</a> bis <a href="Quart%C3%A4rstruktur" title="Quartärstruktur">Quartärstruktur</a>
</td>
<td colspan="2" style="text-align:center;">Heterodimer
</td></tr>
<tr>
<td><a href="Koenzym" class="mw-redirect" title="Koenzym">Kofaktor</a>
</td>
<td colspan="2" style="text-align:center;">Ca<sup>2+</sup>
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<tr>
<td><a href="Isoform" title="Isoform">Isoformen</a>
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<td colspan="2" style="text-align:center;">A, B
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<tr>
<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>
</td>
<td colspan="2" class="" style="text-align:center;"><i><a rel="nofollow" class="external text" href="https://www.genenames.org/tools/search/#!/all?query=3544">F7</a></i>
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<tr>
<td>Externe IDs
</td>
<td colspan="2" class="">
<ul><li><a href="Online_Mendelian_Inheritance_in_Man" title="Online Mendelian Inheritance in Man">OMIM</a>:&nbsp;<a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/omim/227500">227500</a></li>
<li><a href="UniProt" title="UniProt">UniProt</a> <a rel="nofollow" class="external text" href="https://www.uniprot.org/uniprotkb/P08709">P08709</a></li>
<li><a href="Mouse_Genome_Informatics" title="Mouse Genome Informatics">MGI</a>:&nbsp;<a rel="nofollow" class="external text" href="https://www.informatics.jax.org/marker/MGI:109325">109325</a></li>
<li><a href="CAS-Nummer" title="CAS-Nummer">CAS-Nummer</a>:&nbsp;<span title="Untervorlage eingebunden: CASRN"></span><a rel="nofollow" class="external text" href="https://commonchemistry.cas.org/detail?cas_rn=9001-25-6">9001-25-6</a><span class="editoronly" style="display:none;"></span></li></ul>
</td></tr>

<tr>
<th colspan="3" style="background:#90EE90; color:#202122;">Arzneistoffangaben
</th></tr>
<tr>
<td><a href="Anatomisch-Therapeutisch-Chemisches_Klassifikationssystem" title="Anatomisch-Therapeutisch-Chemisches Klassifikationssystem">ATC-Code</a>
</td>
<td colspan="2" style="text-align:center;">B02<a rel="nofollow" class="external text" href="https://atcddd.fhi.no/atc_ddd_index/?code=B02BD01">BD01</a><span class="editoronly" style="display:none;"></span><br>B02<a rel="nofollow" class="external text" href="https://atcddd.fhi.no/atc_ddd_index/?code=B02BD05">BD05</a><span class="editoronly" style="display:none;"></span>
</td></tr>
<tr>
<td><a href="DrugBank" title="DrugBank">DrugBank</a>
</td>
<td colspan="2" class="" style="text-align:center;"><a rel="nofollow" class="external text" href="https://www.drugbank.ca/drugs/DB00036">DB00036</a>
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<tr>
<td>Wirkstoffklasse
</td>
<td colspan="2" style="text-align:center;"><a href="Antih%C3%A4morrhagika" class="mw-redirect" title="Antihämorrhagika">Antihämorrhagika</a>
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<tr>
<th colspan="3" style="background:#90EE90; color:#202122;">Enzymklassifikation
</th></tr>
<tr>
<td><a href="EC-Nummer" title="EC-Nummer">EC, Kategorie</a>
</td>
<td colspan="2" class="" style="text-align:center;"><a rel="nofollow" class="external text" href="https://www.brenda-enzymes.org/enzyme.php?ecno=3.4.21.21">3.4.21.21</a>,&nbsp;<a href="Serinprotease" class="mw-redirect" title="Serinprotease">Serinprotease</a>
</td></tr>
<tr>
<td><a href="MEROPS" title="MEROPS">MEROPS</a>
</td>
<td colspan="2" class="" style="text-align:center;"><a rel="nofollow" class="external text" href="https://www.ebi.ac.uk/merops/cgi-bin/pepsum?id=S01.215">S01.215</a>
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<tr>
<td>Substrat
</td>
<td colspan="2" style="text-align:center;">Arg-+-Ile in Faktor X
</td></tr>
<tr>
<td>Produkte
</td>
<td colspan="2" style="text-align:center;">Faktor Xa
</td></tr>

<tr>
<th colspan="3" style="background:#90EE90; color:#202122;">Vorkommen
</th></tr>
<tr>
<td style="background:#C3FDB8; color:#202122;">Homologie-Familie
</td>
<td colspan="2" style="text-align:center;"><a rel="nofollow" class="external text" href="http://hogenom.univ-lyon1.fr/query_sequence?seq=P08709">Serinprotease</a>
</td></tr>
<tr>
<td style="background:#C3FDB8; color:#202122;">Übergeordnetes <a href="Taxon" title="Taxon">Taxon</a>
</td>
<td colspan="2" style="text-align:center;"><a href="Euteleostomi" title="Euteleostomi">Euteleostomi</a>
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<tr>
<td colspan="3" style="background:#90EE90; color:#202122; text-align:center;"><a href="Homologie_(Genetik)#Homologie_zwischen_verdoppelten_oder_fremden_Genen" title="Homologie (Genetik)">Orthologe</a>
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<td style="background:#C3FDB8; color:#202122;">
</td>
<td style="background:#C3FDB8; color:#202122; text-align:center;">Mensch
</td>
<td style="background:#C3FDB8; color:#202122; text-align:center;">Hausmaus
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<tr>
<td style="background:#C3FDB8; color:#202122;"><a href="Entrez_Gene" title="Entrez Gene">Entrez</a>
</td>
<td><span class=""><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=gene&amp;cmd=retrieve&amp;dopt=default&amp;list_uids=2155&amp;rn=1">2155</a></span>
</td>
<td><span class=""><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=gene&amp;cmd=retrieve&amp;dopt=default&amp;list_uids=14068&amp;rn=1">14068</a></span>
</td></tr>
<tr>
<td style="background:#C3FDB8; color:#202122;"><a href="Ensembl" title="Ensembl">Ensembl</a>
</td>
<td><span class=""><small><small><a rel="nofollow" class="external text" href="http://www.ensembl.org/Homo_sapiens/geneview?gene=ENSG00000057593;db=core">ENSG00000057593</a></small></small></span>
</td>
<td><span class=""><small><small><a rel="nofollow" class="external text" href="http://www.ensembl.org/Mus_musculus/geneview?gene=ENSMUSG00000031443;db=core">ENSMUSG00000031443</a></small></small></span>
</td></tr>
<tr>
<td style="background:#C3FDB8; color:#202122;"><a href="UniProt" title="UniProt">UniProt</a>
</td>
<td><a rel="nofollow" class="external text" href="https://www.uniprot.org/uniprotkb/P08709">P08709</a>
</td>
<td><a rel="nofollow" class="external text" href="https://www.uniprot.org/uniprotkb/P70375">P70375</a>
</td></tr>
<tr>
<td style="background:#C3FDB8; color:#202122;"><a href="National_Center_for_Biotechnology_Information" title="National Center for Biotechnology Information">Refseq</a> (mRNA)
</td>
<td><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/entrez/viewer.fcgi?val=NM_000131">NM_000131</a>
</td>
<td><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/entrez/viewer.fcgi?val=NM_010172">NM_010172</a>
</td></tr>
<tr>
<td style="background:#C3FDB8; color:#202122;"><a href="National_Center_for_Biotechnology_Information" title="National Center for Biotechnology Information">Refseq</a> (Protein)
</td>
<td><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/entrez/viewer.fcgi?val=NP_000122">NP_000122</a>
</td>
<td><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/entrez/viewer.fcgi?val=NP_034302">NP_034302</a>
</td></tr>
<tr>
<td style="background:#C3FDB8; color:#202122;"><a href="Genlocus" title="Genlocus">Genlocus</a>
</td>
<td><span class=""> <a rel="nofollow" class="external text" href="https://genome.ucsc.edu/cgi-bin/hgTracks?org=Human?db=hg38&amp;position=chr13:113105788-113120681">Chr 13: 113.11 – 113.12 Mb</a> </span>
</td>
<td><span class=""> <a rel="nofollow" class="external text" href="https://genome.ucsc.edu/cgi-bin/hgTracks?db=mm10&amp;position=chr8:13026034-13035809">Chr 8: 13.03 – 13.04 Mb</a> </span>
</td></tr>
<tr>
<td style="background:#C3FDB8; color:#202122;"><a href="PubMed" title="PubMed">PubMed</a>-Suche
</td>
<td><span class=""><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&amp;cmd=Link&amp;LinkName=gene_pubmed&amp;from_uid=2155">2155</a></span>
</td>
<td><span class=""><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&amp;cmd=Link&amp;LinkName=gene_pubmed&amp;from_uid=14068">14068</a></span>
<p><span class="editoronly" style="display:none;"></span>
</p>
</td></tr></tbody></table><p><span class="editoronly" style="display:none;"></span>
</p><p><b>Prokonvertin</b> (engl.: <i>Proconvertin</i> oder <i>serum prothrombin conversion accelerator</i> (SPCA); <a href="Synonym" title="Synonym">Synonyme</a>: <i>stabiler Faktor</i>, <i>Prothrombinogen</i> und <b>Gerinnungsfaktor VII</b>) ist ein an der <a href="H%C3%A4mostase" title="Hämostase">Blutgerinnung</a> beteiligtes <a href="Enzym" title="Enzym">Enzym</a>. Die Synthese findet in der Leber statt und ist abhängig von <a href="Phyllochinon" title="Phyllochinon">Vitamin K</a>. Proconvertin hat eine <a href="Molek%C3%BClmasse" title="Molekülmasse">Molekülmasse</a> von 59&nbsp;<a href="Dalton_(Einheit)" class="mw-redirect" title="Dalton (Einheit)">kDa</a> und gehört zur Klasse der <a href="Globuline" title="Globuline">β-Globuline</a>.
</p>

<div class="mw-heading mw-heading2"><h2 id="Genetik">Genetik</h2></div>
<p>Das Gen für Proconvertin liegt auf <a href="Chromosom_13_(Mensch)" title="Chromosom 13 (Mensch)">Chromosom 13</a> (13q34).
</p>
<div class="mw-heading mw-heading2"><h2 id="Physiologie">Physiologie</h2></div>
<p>Die Hauptfunktion von Proconvertin ist die Aktivierung des Gerinnungsprozesses. Kommt es zu einer Verletzung der Blutgefäße, so gelangt der im Gewebe vorhandene <a href="Gewebefaktor" title="Gewebefaktor">Gewebefaktor</a> (Faktor III, Gewebsthrombokinase, Gewebethromboplastin) ins Blut und aktiviert dort, zusammen mit <a href="Calcium" title="Calcium">Calcium</a>, Proconvertin. Dieses aktivierte Proconvertin (Faktor VIIa) aktiviert nun, zusammen mit Calcium und <a href="Phospholipid" class="mw-redirect" title="Phospholipid">Phospholipiden</a>, <a href="Stuart-Prower-Faktor" title="Stuart-Prower-Faktor">Faktor X</a> (Stuart-Prower-Faktor) und <a href="Christmas-Faktor" title="Christmas-Faktor">Faktor IX</a> (Christmas-Faktor).
</p>
<div class="mw-heading mw-heading2"><h2 id="Erkrankungen">Erkrankungen</h2></div>
<p>Erkrankungen, deren Ursache eine <a href="Mutation" title="Mutation">Mutation</a> des Proconvertins ist, sind selten (1 auf 500.000 in der Allgemeinbevölkerung) und werden <a href="Autosomal" class="mw-redirect" title="Autosomal">autosomal</a> <a href="Rezessiv" title="Rezessiv">rezessiv</a> vererbt.
</p>
<div class="mw-heading mw-heading2"><h2 id="Therapeutische_Verwendung">Therapeutische Verwendung</h2></div>
<p><a href="Rekombinantes_Protein" title="Rekombinantes Protein">Rekombinantes</a> Proconvertin (im Präparat <i>NovoSeven</i>) wurde als therapeutische Maßnahme bei unkontrollierbaren Blutungen in <a href="H%C3%A4mophilie" title="Hämophilie">Hämophilie</a>-Patienten vorgestellt, die Hemmer gegen Ersatzgerinnungsfaktoren gebildet hatten.
</p><p>Es kann bei unkontrollierbaren Blutungen verwendet werden.<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> Die Hoffnung dabei ist, dass es nur dort die Blutgerinnung einleitet, wo bereits Thromboplastin vorhanden ist. Studien belegten jedoch ein erhöhtes <a href="Thrombose" title="Thrombose">Thromboserisiko</a> der tiefen <a href="Vene" title="Vene">Venen</a>, <a href="Lungenembolie" title="Lungenembolie">Lungenembolien</a> und <a href="Herzinfarkt" title="Herzinfarkt">Herzinfarkte</a> bei der Verwendung von rekombinantem Faktor VIIa.<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> Ein weiterer Nachteil dieser Therapie ist ihr extrem hoher Preis.
</p>
<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">Harold R. Roberts, Dougald M. Monroe, Gilbert C. White: <i>The use of recombinant factor VIIa in the treatment of bleeding disorders.</i> In: <i><a href="Blood_(Zeitschrift)" title="Blood (Zeitschrift)">Blood</a></i>, Bd. 104 (2004), Heft 13, S. 3858–3864, <a class="external mw-magiclink-pmid" rel="nofollow" href="https://www.ncbi.nlm.nih.gov/pubmed/15328151?dopt=Abstract">PMID 15328151</a>.</span>
</li>
<li id="cite_note-2"><span class="mw-cite-backlink"><a href="#cite_ref-2">↑</a></span> <span class="reference-text">Kathryn A. O’Connell, Jennifer J. Wood, Robert P. Wise, Jay N. Lozier, M. Miles Braun: <i>Thromboembolic adverse events after use of recombinant human coagulation factor VIIa.</i> In: <i>The Journal of the <a href="American_Medical_Association" title="American Medical Association">American Medical Association</a></i> (JAMA), Bd. 295 (2006), S. 293–298.</span>
</li>
</ol>
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<div class="klappleiste-kopf"><a href="Gerinnungsfaktor" class="mw-redirect" title="Gerinnungsfaktor">Gerinnungsfaktoren</a></div>
<div class="klappleiste-inhalt mw-collapsible-content">
<p><b>I</b> <a href="Fibrinogen" title="Fibrinogen">Fibrinogen</a>&nbsp;|
<b>II</b> <a href="Thrombin" title="Thrombin">Prothrombin</a>&nbsp;|
<b>III</b> <a href="Gewebefaktor" title="Gewebefaktor">Gewebefaktor</a>&nbsp;|
<b>IV</b> <a href="Calcium" title="Calcium">Calcium</a>&nbsp;|
<b>V</b> <a href="Proaccelerin" title="Proaccelerin">Proaccelerin</a>&nbsp;|
<b>VI</b> <i>Nicht vergeben</i>&nbsp;|
<b>VII</b> <a class="mw-selflink selflink">Proconvertin</a>&nbsp;|
<b>VIII</b> <a href="Blutgerinnungsfaktor_VIII" title="Blutgerinnungsfaktor VIII">Blutgerinnungsfaktor VIII</a>&nbsp;|
<b>IX</b> <a href="Christmas-Faktor" title="Christmas-Faktor">Christmas-Faktor</a>&nbsp;|
<b>X</b> <a href="Stuart-Prower-Faktor" title="Stuart-Prower-Faktor">Stuart-Prower-Faktor</a>&nbsp;|
<b>XI</b> <a href="Plasma_Thromboplastin_Antecedent" title="Plasma Thromboplastin Antecedent">PTA (Plasma Thromboplastin Antecedent)</a>&nbsp;|
<b>XII</b> <a href="Hageman-Faktor" title="Hageman-Faktor">Hageman-Faktor</a>&nbsp;|
<b>XIII</b> <a href="Fibrinstabilisierender_Faktor" title="Fibrinstabilisierender Faktor">Fibrinstabilisierender Faktor</a>
</p><p><i>Weitere Gerinnungsfaktoren:</i> <a href="Kallikrein" title="Kallikrein">Präkallikrein</a>&nbsp;|&nbsp;<a href="Kininogen" class="mw-redirect" title="Kininogen">Hochmolekulares Kininogen</a>
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