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

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<th colspan="3" style="background:#90EE90; color:#202122;">Fumarase
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<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;">Modell des homotetramer der Fumarase, E.Coli 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/1FUO">1FUO</a>
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<tr>
<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;">466 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;">Tetramer
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<tr>
<td><a href="Isoform" title="Isoform">Isoformen</a>
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<td colspan="2" style="text-align:center;">2
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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=3700">FH</a></i>
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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>:&nbsp;<a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/omim/136850">136850</a></li>
<li><a href="UniProt" title="UniProt">UniProt</a> <a rel="nofollow" class="external text" href="https://www.uniprot.org/uniprotkb/P07954">P07954</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=9032-88-6">9032-88-6</a><span class="editoronly" style="display:none;"></span></li></ul>
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<th colspan="3" style="background:#90EE90; color:#202122;">Enzymklassifikation
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<td><a href="EC-Nummer" title="EC-Nummer">EC, Kategorie</a>
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<td colspan="2" class="" style="text-align:center;"><a rel="nofollow" class="external text" href="https://www.brenda-enzymes.org/enzyme.php?ecno=4.2.1.2">4.2.1.2</a>,&nbsp;<a href="Lyase" class="mw-redirect" title="Lyase">Lyase</a>
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<td>Reaktionsart
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<td colspan="2" style="text-align:center;">Hydratation
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<td>Substrat
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<td colspan="2" style="text-align:center;"><small>L</small>-Malat; Fumarat + H<sub>2</sub>O
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<td>Produkte
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<td colspan="2" style="text-align:center;">Fumarat + H<sub>2</sub>O; <small>L</small>-Malat
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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=P07954">Fumarase II</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;">Lebewesen
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<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;">
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<td style="background:#C3FDB8; color:#202122; text-align:center;">Mensch
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<td style="background:#C3FDB8; color:#202122; text-align:center;">Hausmaus
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<td style="background:#C3FDB8; color:#202122;"><a href="Entrez_Gene" title="Entrez Gene">Entrez</a>
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<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=2271&amp;rn=1">2271</a></span>
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<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=14194&amp;rn=1">14194</a></span>
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<td style="background:#C3FDB8; color:#202122;"><a href="Ensembl" title="Ensembl">Ensembl</a>
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<td><span class=""><small><small><a rel="nofollow" class="external text" href="http://www.ensembl.org/Homo_sapiens/geneview?gene=ENSG00000091483;db=core">ENSG00000091483</a></small></small></span>
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<td><span class=""><small><small><a rel="nofollow" class="external text" href="http://www.ensembl.org/Mus_musculus/geneview?gene=ENSMUSG00000026526;db=core">ENSMUSG00000026526</a></small></small></span>
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<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/P07954">P07954</a>
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<td><a rel="nofollow" class="external text" href="https://www.uniprot.org/uniprotkb/P97807">P97807</a>
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<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_000143">NM_000143</a>
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<td><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/entrez/viewer.fcgi?val=NM_010209">NM_010209</a>
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<td style="background:#C3FDB8; color:#202122;"><a href="National_Center_for_Biotechnology_Information" title="National Center for Biotechnology Information">Refseq</a> (Protein)
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<td><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/entrez/viewer.fcgi?val=NP_000134">NP_000134</a>
</td>
<td><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/entrez/viewer.fcgi?val=NP_034339">NP_034339</a>
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<td style="background:#C3FDB8; color:#202122;"><a href="Genlocus" title="Genlocus">Genlocus</a>
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<td><span class=""> <a rel="nofollow" class="external text" href="https://genome.ucsc.edu/cgi-bin/hgTracks?org=Human?db=hg38&amp;position=chr1:241497603-241519761">Chr 1: 241.5 – 241.52 Mb</a> </span>
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<td><span class=""> <a rel="nofollow" class="external text" href="https://genome.ucsc.edu/cgi-bin/hgTracks?db=mm10&amp;position=chr1:175600374-175625635">Chr 1: 175.6 – 175.63 Mb</a> </span>
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<td style="background:#C3FDB8; color:#202122;"><a href="PubMed" title="PubMed">PubMed</a>-Suche
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<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=2271">2271</a></span>
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<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=14194">14194</a></span>
<p><span class="editoronly" style="display:none;"></span>
</p>
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</p><p><b>Fumarase</b> (genauer: <b>Fumarat-Hydratase</b>) heißen <a href="Enzym" title="Enzym">Enzyme</a>, welche die <a href="Additionsreaktion" title="Additionsreaktion">Addition</a> von Wasser an <a href="Fumarat" class="mw-redirect" title="Fumarat">Fumarat</a> zum <a href="Malat" class="mw-redirect" title="Malat"><small>L</small>-Malat</a> bzw. die zugehörige <a href="R%C3%BCckreaktion" class="mw-redirect" title="Rückreaktion">Rückreaktion</a> <a href="Katalyse" title="Katalyse">katalysieren</a>. Diese Reaktion ist unentbehrlich im <a href="Citratzyklus" title="Citratzyklus">Citratzyklus</a>.
</p>
<dl><dd><span typeof="mw:File"></span> + H<sub>2</sub>O &nbsp; <span class="mwe-math-element mwe-math-element-inline"><span class="mwe-math-mathml-inline mwe-math-mathml-a11y" style="display: none;"><math xmlns="http://www.w3.org/1998/Math/MathML" alttext="{\displaystyle \rightleftharpoons }">
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<annotation encoding="application/x-tex">{\displaystyle \rightleftharpoons }</annotation>
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</math></span><img src="./_assets_/eb734a37dd21ce173a46342d1cc64c92/1c37b981df851b9e54e489e017b1481e37d418f3.svg" class="mwe-math-fallback-image-inline mw-invert skin-invert" aria-hidden="true" style="vertical-align: -0.338ex; width:2.324ex; height:1.843ex;" alt="{\displaystyle \rightleftharpoons }" loading="lazy"></span> &nbsp; <span typeof="mw:File"></span></dd></dl>
<p>Es gibt zwei verschiedene Klassen von Fumarasen: Fumarasen I sind bakterielle eisenclusterhaltige, hitzelabile Proteine, während Fumarasen II in <a href="Prokaryoten" title="Prokaryoten">Prokaryoten</a> und <a href="Eukaryoten" title="Eukaryoten">Eukaryoten</a> vorkommen, für ihre katalytische Wirkung keine Metallionen benötigen und wesentlich beständiger sind. Fumarasen I treten als Dimer auf, Fumarasen II als Tetramer in den <a href="Mitochondrium" title="Mitochondrium">Mitochondrien</a>.
</p><p>Mangel an menschlicher Fumarase II kann durch <a href="Mutation" title="Mutation">Mutation</a> des <i>FH</i>-<a href="Gen" title="Gen">Gens</a> entstehen und zur <a href="Fumarazidurie" title="Fumarazidurie">Fumarazidurie</a><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> und Neigung zu <a href="Myom" title="Myom">Myomen</a> führen.<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><sup id="cite_ref-3" class="reference"><a href="#cite_note-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup>
</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">Eintrag zu <a rel="nofollow" class="external text" href="https://www.orpha.net/de/disease/detail/24"><i>Fumarazidurie.</i></a> In: <i><a href="Orphanet" title="Orphanet">Orphanet</a></i> (Datenbank für seltene Krankheiten)<span class="editoronly" style="display:none;"></span></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="http://www.expasy.org/prosite/PDOC00147">PROSITE-Eintrag</a></span>
</li>
<li id="cite_note-3"><span class="mw-cite-backlink"><a href="#cite_ref-3">↑</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/P07954">P07954</a></span>
</li>
</ol>
<div class="mw-heading mw-heading2"><h2 id="Weblinks">Weblinks</h2></div>
<div class="sisterproject" style="margin:0.1em 0 0 0;"><div class="noviewer" style="display:inline-block; line-height:10px; min-width:1.6em; text-align:center;" aria-hidden="true" role="presentation"><span class="mw-default-size" typeof="mw:File"><span title="Wikibooks"></span></span></div><b><a href="https://de.wikibooks.org/wiki/Biochemie_und_Pathobiochemie:_Fumarase" class="extiw external" title="b:Biochemie und Pathobiochemie: Fumarase">Wikibooks: Biochemie und Pathobiochemie: Fumarase</a></b>&nbsp;– Lern- und Lehrmaterialien</div></div><!--htdig_noindex--><div><div class="zim-footer">
Dieser Artikel wurde von <a class="external text" title="Zuletzt bearbeitet am 2024-07-26" href="https://de.wikipedia.org/wiki/?title=Fumarase&amp;oldid=247097280">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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