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margin-top:0; width:350px;" summary="Infobox Protein"> <tbody><tr> <th colspan="3" style="background:#90EE90; color:#202122;">Carbamoylphosphat-Synthetase I </th></tr> <tr> <td>Andere Namen </td> <td colspan="2"> <ul><li>Carbamoylphosphat-Synthase I</li> <li>CPSase I</li></ul> </td></tr> <tr> <td colspan="3" class="hintergrundfarbe1" style="font-size:smaller;"> <p>Vorhandene Strukturdaten: <span class="plainlinks"><a rel="nofollow" class="external text" href="https://www.rcsb.org/structure/2YVQ">2YVQ</a></span>, <span class="plainlinks"><a rel="nofollow" class="external text" href="https://www.rcsb.org/structure/5DOT">5DOT</a></span>, <span class="plainlinks"><a rel="nofollow" class="external text" href="https://www.rcsb.org/structure/5DOU">5DOU</a></span> </p> </td></tr> <tr> <th colspan="3" style="background:#90EE90; color:#202122;;">Eigenschaften des menschlichen Proteins </th></tr> <tr> <td><a href="/wiki/Molare_Masse" title="Molare Masse">Masse</a>/Länge <a href="/wiki/Prim%C3%A4rstruktur" title="Primärstruktur">Primärstruktur</a> </td> <td colspan="2" style="text-align:center;">1462 Aminosäuren </td></tr> <tr> <td><a href="/wiki/Koenzym" class="mw-redirect" title="Koenzym">Kofaktor</a> </td> <td colspan="2" style="text-align:center;"><i>N</i>-Acetylglutamat </td></tr> <tr> <td><a href="/wiki/Isoform" title="Isoform">Isoformen</a> </td> <td colspan="2" style="text-align:center;">2 </td></tr> <tr> <th colspan="3" style="background:#90EE90; color:#202122;">Bezeichner </th></tr> <tr> <td><a href="/wiki/Human_Genome_Organisation" title="Human Genome Organisation">Gen-Name</a> </td> <td colspan="2" class="plainlinks" style="text-align:center;"><i><a rel="nofollow" class="external text" href="https://www.genenames.org/tools/search/#!/all?query=2323">CPS1</a></i> </td></tr> <tr> <td>Externe IDs </td> <td colspan="2" class="plainlinks"> <ul><li><a href="/wiki/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/608307">608307</a></li> <li><a href="/wiki/UniProt" title="UniProt">UniProt</a> <a rel="nofollow" class="external text" href="https://www.uniprot.org/uniprotkb/P31327">P31327</a></li></ul> </td></tr> <tr> <th colspan="3" style="background:#90EE90; color:#202122;">Enzymklassifikation </th></tr> <tr> <td><a href="/wiki/EC-Nummer" title="EC-Nummer">EC, Kategorie</a> </td> <td colspan="2" class="plainlinks" style="text-align:center;"><a rel="nofollow" class="external text" href="https://www.brenda-enzymes.org/enzyme.php?ecno=6.3.4.16">6.3.4.16</a>, <a href="/wiki/Ligase" title="Ligase">Ligase</a> </td></tr> <tr> <td>Reaktionsart </td> <td colspan="2" style="text-align:center;">Addition </td></tr> <tr> <td>Substrat </td> <td colspan="2" style="text-align:center;">2 ATP + NH<sub>3</sub> + CO<sub>2</sub> </td></tr> <tr> <td>Produkte </td> <td colspan="2" style="text-align:center;">2 ADP + Phosphat + Carbamoylphosphat </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=P31327">Hovergen</a> </td></tr> <tr> <td style="background:#C3FDB8; color:#202122;">Übergeordnetes <a href="/wiki/Taxon" title="Taxon">Taxon</a> </td> <td colspan="2" style="text-align:center;"><a href="/wiki/Wirbeltiere" title="Wirbeltiere">Wirbeltiere</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> </td></tr> <tr> <td colspan="3" style="background:#90EE90; color:#202122; text-align:center;"><a href="/wiki/Homologie_(Genetik)#Homologie_zwischen_verdoppelten_oder_fremden_Genen" title="Homologie (Genetik)">Orthologe</a> </td></tr> <tr> <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 </td></tr> <tr> <td style="background:#C3FDB8; color:#202122;"><a href="/wiki/Entrez_Gene" title="Entrez Gene">Entrez</a> </td> <td><span class="plainlinks"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=gene&cmd=retrieve&dopt=default&list_uids=1373&rn=1">1373</a></span> </td> <td><span class="plainlinks"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=gene&cmd=retrieve&dopt=default&list_uids=227231&rn=1">227231</a></span> </td></tr> <tr> <td style="background:#C3FDB8; color:#202122;"><a href="/wiki/Ensembl" title="Ensembl">Ensembl</a> </td> <td><span class="plainlinks"><small><small><a rel="nofollow" class="external text" href="http://www.ensembl.org/Homo_sapiens/geneview?gene=ENSG00000021826;db=core">ENSG00000021826</a></small></small></span> </td> <td><span class="plainlinks"><small><small><a rel="nofollow" class="external text" href="http://www.ensembl.org/Mus_musculus/geneview?gene=ENSMUSG00000025991;db=core">ENSMUSG00000025991</a></small></small></span> </td></tr> <tr> <td style="background:#C3FDB8; color:#202122;"><a href="/wiki/UniProt" title="UniProt">UniProt</a> </td> <td><a rel="nofollow" class="external text" href="https://www.uniprot.org/uniprotkb/P31327">P31327</a> </td> <td><a rel="nofollow" class="external text" href="https://www.uniprot.org/uniprotkb/Q8C196">Q8C196</a> </td></tr> <tr> <td style="background:#C3FDB8; color:#202122;"><a href="/wiki/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_001122633">NM_001122633</a> </td> <td><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/entrez/viewer.fcgi?val=NM_001080809">NM_001080809</a> </td></tr> <tr> <td style="background:#C3FDB8; color:#202122;"><a href="/wiki/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_001116105">NP_001116105</a> </td> <td><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/entrez/viewer.fcgi?val=NP_001074278">NP_001074278</a> </td></tr> <tr> <td style="background:#C3FDB8; color:#202122;"><a href="/wiki/Genlocus" title="Genlocus">Genlocus</a> </td> <td><span class="plainlinks"> <a rel="nofollow" class="external text" href="https://genome.ucsc.edu/cgi-bin/hgTracks?org=Human?db=hg38&position=chr2:210477682-210679107">Chr 2: 210.48 – 210.68 Mb</a> </span> </td> <td><span class="plainlinks"> <a rel="nofollow" class="external text" href="https://genome.ucsc.edu/cgi-bin/hgTracks?db=mm10&position=chr1:67122426-67231267">Chr 1: 67.12 – 67.23 Mb</a> </span> </td></tr> <tr> <td style="background:#C3FDB8; color:#202122;"><a href="/wiki/PubMed" title="PubMed">PubMed</a>-Suche </td> <td><span class="plainlinks"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Link&LinkName=gene_pubmed&from_uid=1373">1373</a></span> </td> <td><span class="plainlinks"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Link&LinkName=gene_pubmed&from_uid=227231">227231</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>Carbamoylphosphat-Synthetase I (CPS1)</b> ist das <a href="/wiki/Enzym" title="Enzym">Enzym</a>, das die <a href="/wiki/Additionsreaktion" title="Additionsreaktion">Addition</a> von <a href="/wiki/Ammoniak" title="Ammoniak">Ammoniak</a>, <a href="/wiki/Kohlenstoffdioxid" title="Kohlenstoffdioxid">Kohlenstoffdioxid</a> und <a href="/wiki/Adenosintriphosphat" title="Adenosintriphosphat">ATP</a> zu <a href="/wiki/Carbamoylphosphat" title="Carbamoylphosphat">Carbamoylphosphat</a> <a href="/wiki/Katalyse" title="Katalyse">katalysiert</a>. Diese Reaktion findet in den <a href="/wiki/Mitochondrium" title="Mitochondrium">Mitochondrien</a> der <a href="/wiki/Wirbeltiere" title="Wirbeltiere">Wirbeltiere</a> statt und ist der geschwindigkeitsbestimmende Schritt des <a href="/wiki/Harnstoffzyklus" title="Harnstoffzyklus">Harnstoffzyklus</a>. Beim Menschen ist CPS1 hauptsächlich in der <a href="/wiki/Leber" title="Leber">Leber</a> und im <a href="/wiki/D%C3%BCnndarm" title="Dünndarm">Dünndarm</a> aktiv. <a href="/wiki/Mutation" title="Mutation">Mutationen</a> im <i>CPS1</i>-<a href="/wiki/Gen" title="Gen">Gen</a> können zu CPS-Mangel und dieser zu <a href="/wiki/Hyperammon%C3%A4mie" title="Hyperammonämie">Hyperammonämie</a> und <a href="/wiki/Pulmonale_Hypertonie" title="Pulmonale Hypertonie">pulmonaler Hypertonie</a> des Neugeborenen führen. Eine Variante ist mit dem Risiko bei Neugeborenen für <a href="/wiki/Arginin" title="Arginin">Argininmangel</a> und <a href="/wiki/Nekrotisierende_Enterokolitis" title="Nekrotisierende Enterokolitis">nekrotisierende Enterokolitis</a> assoziiert.<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><p>Die CPS1-Transkription wird sowohl über <a href="/wiki/Cyclisches_Adenosinmonophosphat" title="Cyclisches Adenosinmonophosphat">cAMP</a>-, als auch <a href="/wiki/Glucocorticoid" class="mw-redirect" title="Glucocorticoid">Glucocorticoid</a>-<a href="/wiki/Signalweg" class="mw-redirect" title="Signalweg">Signalwege</a> gesteuert. Die Expression von CPS1 ist in den drei menschlichen <a href="/wiki/Leberkarzinom" class="mw-redirect" title="Leberkarzinom">Leberkarzinom</a>-<a href="/wiki/Zelllinie" title="Zelllinie">Zelllinien</a> unterdrückt. CPS1 ist mit dem Antigen des in der Chirurgie eingesetzten <a href="/w/index.php?title=Hep_Par_1&action=edit&redlink=1" class="new" title="Hep Par 1 (Seite nicht vorhanden)">Hep Par 1</a>-Antikörpers identisch.<sup id="cite_ref-4" class="reference"><a href="#cite_note-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-5" class="reference"><a href="#cite_note-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Katalysierte_Reaktion">Katalysierte Reaktion</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Carbamoylphosphat-Synthetase_I&veaction=edit&section=1" title="Abschnitt bearbeiten: Katalysierte Reaktion" class="mw-editsection-visualeditor"><span>Bearbeiten</span></a><span class="mw-editsection-divider"> | </span><a href="/w/index.php?title=Carbamoylphosphat-Synthetase_I&action=edit&section=1" title="Quellcode des Abschnitts bearbeiten: Katalysierte Reaktion"><span>Quelltext bearbeiten</span></a><span class="mw-editsection-bracket">]</span></span></div> <dl><dd><span class="mwe-math-element"><span class="mwe-math-mathml-inline mwe-math-mathml-a11y" style="display: none;"><math xmlns="http://www.w3.org/1998/Math/MathML" alttext="{\displaystyle \mathrm {HCO_{3}^{-}+ATP\longrightarrow } }"> <semantics> <mrow class="MJX-TeXAtom-ORD"> <mstyle displaystyle="true" scriptlevel="0"> <mrow class="MJX-TeXAtom-ORD"> <mi mathvariant="normal">H</mi> <mi mathvariant="normal">C</mi> <msubsup> <mi mathvariant="normal">O</mi> <mrow class="MJX-TeXAtom-ORD"> <mn>3</mn> </mrow> <mrow class="MJX-TeXAtom-ORD"> <mo>−</mo> </mrow> </msubsup> <mo>+</mo> <mi mathvariant="normal">A</mi> <mi mathvariant="normal">T</mi> <mi mathvariant="normal">P</mi> <mo stretchy="false">⟶</mo> </mrow> </mstyle> </mrow> <annotation encoding="application/x-tex">{\displaystyle \mathrm {HCO_{3}^{-}+ATP\longrightarrow } }</annotation> </semantics> </math></span><img src="https://wikimedia.org/api/rest_v1/media/math/render/svg/61e7f2951b1effb8af2f6d7e5597f4b3f8455b54" class="mwe-math-fallback-image-inline mw-invert skin-invert" aria-hidden="true" style="vertical-align: -1.005ex; width:19.035ex; height:3.176ex;" alt="{\displaystyle \mathrm {HCO_{3}^{-}+ATP\longrightarrow } }"></span> <span class="mwe-math-element"><span class="mwe-math-mathml-inline mwe-math-mathml-a11y" style="display: none;"><math xmlns="http://www.w3.org/1998/Math/MathML" alttext="{\displaystyle \mathrm {ADP+HO{-}COO{-}PO_{3}^{2-}} }"> <semantics> <mrow class="MJX-TeXAtom-ORD"> <mstyle displaystyle="true" scriptlevel="0"> <mrow class="MJX-TeXAtom-ORD"> <mi mathvariant="normal">A</mi> <mi mathvariant="normal">D</mi> <mi mathvariant="normal">P</mi> <mo>+</mo> <mi mathvariant="normal">H</mi> <mi mathvariant="normal">O</mi> <mrow class="MJX-TeXAtom-ORD"> <mo>−</mo> </mrow> <mi mathvariant="normal">C</mi> <mi mathvariant="normal">O</mi> <mi mathvariant="normal">O</mi> <mrow class="MJX-TeXAtom-ORD"> <mo>−</mo> </mrow> <mi mathvariant="normal">P</mi> <msubsup> <mi mathvariant="normal">O</mi> <mrow class="MJX-TeXAtom-ORD"> <mn>3</mn> </mrow> <mrow class="MJX-TeXAtom-ORD"> <mn>2</mn> <mo>−</mo> </mrow> </msubsup> </mrow> </mstyle> </mrow> <annotation encoding="application/x-tex">{\displaystyle \mathrm {ADP+HO{-}COO{-}PO_{3}^{2-}} }</annotation> </semantics> </math></span><img src="https://wikimedia.org/api/rest_v1/media/math/render/svg/b25edab04444e59a51a0ba0ce3b3dd4beef0f42b" class="mwe-math-fallback-image-inline mw-invert skin-invert" aria-hidden="true" style="vertical-align: -1.005ex; width:26.128ex; height:3.343ex;" alt="{\displaystyle \mathrm {ADP+HO{-}COO{-}PO_{3}^{2-}} }"></span></dd> <dd><span class="mwe-math-element"><span class="mwe-math-mathml-inline mwe-math-mathml-a11y" style="display: none;"><math xmlns="http://www.w3.org/1998/Math/MathML" alttext="{\displaystyle \mathrm {HO{-}COO{-}PO_{3}^{2-}+NH_{3}+OH^{-}\longrightarrow } }"> <semantics> <mrow class="MJX-TeXAtom-ORD"> <mstyle displaystyle="true" scriptlevel="0"> <mrow class="MJX-TeXAtom-ORD"> <mi mathvariant="normal">H</mi> <mi mathvariant="normal">O</mi> <mrow class="MJX-TeXAtom-ORD"> <mo>−</mo> </mrow> <mi mathvariant="normal">C</mi> <mi mathvariant="normal">O</mi> <mi mathvariant="normal">O</mi> <mrow class="MJX-TeXAtom-ORD"> <mo>−</mo> </mrow> <mi mathvariant="normal">P</mi> <msubsup> <mi mathvariant="normal">O</mi> <mrow class="MJX-TeXAtom-ORD"> <mn>3</mn> </mrow> <mrow class="MJX-TeXAtom-ORD"> <mn>2</mn> <mo>−</mo> </mrow> </msubsup> <mo>+</mo> <mi mathvariant="normal">N</mi> <msub> <mi mathvariant="normal">H</mi> <mrow class="MJX-TeXAtom-ORD"> <mn>3</mn> </mrow> </msub> <mo>+</mo> <mi mathvariant="normal">O</mi> <msup> <mi mathvariant="normal">H</mi> <mrow class="MJX-TeXAtom-ORD"> <mo>−</mo> </mrow> </msup> <mo stretchy="false">⟶</mo> </mrow> </mstyle> </mrow> <annotation encoding="application/x-tex">{\displaystyle \mathrm {HO{-}COO{-}PO_{3}^{2-}+NH_{3}+OH^{-}\longrightarrow } }</annotation> </semantics> </math></span><img src="https://wikimedia.org/api/rest_v1/media/math/render/svg/df66eebb057bc37ace77887fa09e769888dc0aa2" class="mwe-math-fallback-image-inline mw-invert skin-invert" aria-hidden="true" style="vertical-align: -1.005ex; width:37.92ex; height:3.343ex;" alt="{\displaystyle \mathrm {HO{-}COO{-}PO_{3}^{2-}+NH_{3}+OH^{-}\longrightarrow } }"></span> <span class="mwe-math-element"><span class="mwe-math-mathml-inline mwe-math-mathml-a11y" style="display: none;"><math xmlns="http://www.w3.org/1998/Math/MathML" alttext="{\displaystyle \mathrm {HPO_{4}^{2-}+H_{2}N{-}COO^{-}+H_{2}O} }"> <semantics> <mrow class="MJX-TeXAtom-ORD"> <mstyle displaystyle="true" scriptlevel="0"> <mrow class="MJX-TeXAtom-ORD"> <mi mathvariant="normal">H</mi> <mi mathvariant="normal">P</mi> <msubsup> <mi mathvariant="normal">O</mi> <mrow class="MJX-TeXAtom-ORD"> <mn>4</mn> </mrow> <mrow class="MJX-TeXAtom-ORD"> <mn>2</mn> <mo>−</mo> </mrow> </msubsup> <mo>+</mo> <msub> <mi mathvariant="normal">H</mi> <mrow class="MJX-TeXAtom-ORD"> <mn>2</mn> </mrow> </msub> <mi mathvariant="normal">N</mi> <mrow class="MJX-TeXAtom-ORD"> <mo>−</mo> </mrow> <mi mathvariant="normal">C</mi> <mi mathvariant="normal">O</mi> <msup> <mi mathvariant="normal">O</mi> <mrow class="MJX-TeXAtom-ORD"> <mo>−</mo> </mrow> </msup> <mo>+</mo> <msub> <mi mathvariant="normal">H</mi> <mrow class="MJX-TeXAtom-ORD"> <mn>2</mn> </mrow> </msub> <mi mathvariant="normal">O</mi> </mrow> </mstyle> </mrow> <annotation encoding="application/x-tex">{\displaystyle \mathrm {HPO_{4}^{2-}+H_{2}N{-}COO^{-}+H_{2}O} }</annotation> </semantics> </math></span><img src="https://wikimedia.org/api/rest_v1/media/math/render/svg/3d2a8424c07d4ee6623e40c9581751c6f22c4392" class="mwe-math-fallback-image-inline mw-invert skin-invert" aria-hidden="true" style="vertical-align: -1.005ex; width:30.907ex; height:3.343ex;" alt="{\displaystyle \mathrm {HPO_{4}^{2-}+H_{2}N{-}COO^{-}+H_{2}O} }"></span></dd> <dd><span class="mwe-math-element"><span class="mwe-math-mathml-inline mwe-math-mathml-a11y" style="display: none;"><math xmlns="http://www.w3.org/1998/Math/MathML" alttext="{\displaystyle \mathrm {H_{2}N{-}COO^{-}+ATP\longrightarrow } }"> <semantics> <mrow class="MJX-TeXAtom-ORD"> <mstyle displaystyle="true" scriptlevel="0"> <mrow class="MJX-TeXAtom-ORD"> <msub> <mi mathvariant="normal">H</mi> <mrow class="MJX-TeXAtom-ORD"> <mn>2</mn> </mrow> </msub> <mi mathvariant="normal">N</mi> <mrow class="MJX-TeXAtom-ORD"> <mo>−</mo> </mrow> <mi mathvariant="normal">C</mi> <mi mathvariant="normal">O</mi> <msup> <mi mathvariant="normal">O</mi> <mrow class="MJX-TeXAtom-ORD"> <mo>−</mo> </mrow> </msup> <mo>+</mo> <mi mathvariant="normal">A</mi> <mi mathvariant="normal">T</mi> <mi mathvariant="normal">P</mi> <mo stretchy="false">⟶</mo> </mrow> </mstyle> </mrow> <annotation encoding="application/x-tex">{\displaystyle \mathrm {H_{2}N{-}COO^{-}+ATP\longrightarrow } }</annotation> </semantics> </math></span><img src="https://wikimedia.org/api/rest_v1/media/math/render/svg/74a2512800fcd23f56c2c4dce8b63563537a603d" class="mwe-math-fallback-image-inline mw-invert skin-invert" aria-hidden="true" style="vertical-align: -0.671ex; width:25.449ex; height:2.843ex;" alt="{\displaystyle \mathrm {H_{2}N{-}COO^{-}+ATP\longrightarrow } }"></span> <span class="mwe-math-element"><span class="mwe-math-mathml-inline mwe-math-mathml-a11y" style="display: none;"><math xmlns="http://www.w3.org/1998/Math/MathML" alttext="{\displaystyle \mathrm {ADP+H_{2}N{-}COO{-}PO_{3}^{2-}} }"> <semantics> <mrow class="MJX-TeXAtom-ORD"> <mstyle displaystyle="true" scriptlevel="0"> <mrow class="MJX-TeXAtom-ORD"> <mi mathvariant="normal">A</mi> <mi mathvariant="normal">D</mi> <mi mathvariant="normal">P</mi> <mo>+</mo> <msub> <mi mathvariant="normal">H</mi> <mrow class="MJX-TeXAtom-ORD"> <mn>2</mn> </mrow> </msub> <mi mathvariant="normal">N</mi> <mrow class="MJX-TeXAtom-ORD"> <mo>−</mo> </mrow> <mi mathvariant="normal">C</mi> <mi mathvariant="normal">O</mi> <mi mathvariant="normal">O</mi> <mrow class="MJX-TeXAtom-ORD"> <mo>−</mo> </mrow> <mi mathvariant="normal">P</mi> <msubsup> <mi mathvariant="normal">O</mi> <mrow class="MJX-TeXAtom-ORD"> <mn>3</mn> </mrow> <mrow class="MJX-TeXAtom-ORD"> <mn>2</mn> <mo>−</mo> </mrow> </msubsup> </mrow> </mstyle> </mrow> <annotation encoding="application/x-tex">{\displaystyle \mathrm {ADP+H_{2}N{-}COO{-}PO_{3}^{2-}} }</annotation> </semantics> </math></span><img src="https://wikimedia.org/api/rest_v1/media/math/render/svg/cc6bdfbd3205051700847f71fdf15e534cf6e3e8" class="mwe-math-fallback-image-inline mw-invert skin-invert" aria-hidden="true" style="vertical-align: -1.005ex; width:27.117ex; height:3.343ex;" alt="{\displaystyle \mathrm {ADP+H_{2}N{-}COO{-}PO_{3}^{2-}} }"></span></dd></dl> <p>Die in drei Teilschritten erfolgende Reaktion setzt insgesamt zwei Moleküle ATP und je ein Molekül Ammoniak und Kohlenstoffdioxid zu zwei Molekülen ADP und je einem Molekül Carbamoylphosphat und Phosphat um. </p> <div class="mw-heading mw-heading2"><h2 id="Weblinks">Weblinks</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Carbamoylphosphat-Synthetase_I&veaction=edit&section=2" title="Abschnitt bearbeiten: Weblinks" class="mw-editsection-visualeditor"><span>Bearbeiten</span></a><span class="mw-editsection-divider"> | </span><a href="/w/index.php?title=Carbamoylphosphat-Synthetase_I&action=edit&section=2" title="Quellcode des Abschnitts bearbeiten: Weblinks"><span>Quelltext bearbeiten</span></a><span class="mw-editsection-bracket">]</span></span></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"><img alt="" src="//upload.wikimedia.org/wikipedia/commons/thumb/f/fa/Wikibooks-logo.svg/16px-Wikibooks-logo.svg.png" decoding="async" width="16" height="16" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/f/fa/Wikibooks-logo.svg/24px-Wikibooks-logo.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/f/fa/Wikibooks-logo.svg/32px-Wikibooks-logo.svg.png 2x" data-file-width="300" data-file-height="300" /></span></span></div><b><a href="https://de.wikibooks.org/wiki/Biochemie_und_Pathobiochemie:_Harnstoffzyklus" class="extiw" title="b:Biochemie und Pathobiochemie: Harnstoffzyklus">Wikibooks: Harnstoffzyklus</a></b> – Lern- und Lehrmaterialien</div> <ul><li>D’Eustachio/Jassal/reactome: <i><a rel="nofollow" class="external text" href="https://reactome.org/content/detail/R-HSA-70555">2 ATP + NH4+ + HCO3- => 2 ADP + orthophosphate + carbamoyl phosphate [mitochondrial]</a></i></li></ul> <div class="mw-heading mw-heading2"><h2 id="Einzelnachweise">Einzelnachweise</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Carbamoylphosphat-Synthetase_I&veaction=edit&section=3" title="Abschnitt bearbeiten: Einzelnachweise" class="mw-editsection-visualeditor"><span>Bearbeiten</span></a><span class="mw-editsection-divider"> | </span><a href="/w/index.php?title=Carbamoylphosphat-Synthetase_I&action=edit&section=3" title="Quellcode des Abschnitts bearbeiten: Einzelnachweise"><span>Quelltext bearbeiten</span></a><span class="mw-editsection-bracket">]</span></span></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 rel="nofollow" class="external text" href="https://omabrowser.org/oma/omagroup/1185730/members/">Homologe bei OMA</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 href="/wiki/UniProt" title="UniProt">UniProt</a> <a rel="nofollow" class="external text" href="https://www.uniprot.org/uniprotkb/P31327">P31327</a></span> </li> <li id="cite_note-3"><span class="mw-cite-backlink"><a href="#cite_ref-3">↑</a></span> <span class="reference-text">Moonen RM, Paulussen AD, Souren NY, Kessels AG, Rubio-Gozalbo ME, Villamor E: <cite style="font-style:italic">Carbamoyl phosphate synthetase polymorphisms as a risk factor for necrotizing enterocolitis</cite>. In: <cite style="font-style:italic"><a href="/wiki/Pediatr_Res" class="mw-redirect" title="Pediatr Res">Pediatr Res</a></cite>. 62. Jahrgang, <span style="white-space:nowrap">Nr.<span style="display:inline-block;width:.2em"> </span>2</span>, August 2007, <span style="white-space:nowrap">S.<span style="display:inline-block;width:.2em"> </span>188–90</span>, <a href="/wiki/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/10.1203/PDR.0b013e3180a0324e">10.1203/PDR.0b013e3180a0324e</a></span>, <a class="external mw-magiclink-pmid" rel="nofollow" href="//www.ncbi.nlm.nih.gov/pubmed/17597649?dopt=Abstract">PMID 17597649</a>.<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:Carbamoylphosphat-Synthetase+I&rft.atitle=Carbamoyl+phosphate+synthetase+polymorphisms+as+a+risk+factor+for+necrotizing+enterocolitis&rft.au=Moonen+RM%2C+Paulussen+AD%2C+Souren+NY%2C+...&rft.date=2007-08&rft.doi=10.1203%2FPDR.0b013e3180a0324e&rft.genre=journal&rft.issue=2&rft.jtitle=Pediatr+Res&rft.pages=188-90&rft.pmid=17597649&rft.volume=62.+Jahrgang" style="display:none"> </span></span> </li> <li id="cite_note-4"><span class="mw-cite-backlink"><a href="#cite_ref-4">↑</a></span> <span class="reference-text">Schoneveld OJ, Hoogenkamp M, Stallen JM, Gaemers IC, Lamers WH: <cite style="font-style:italic">cyclicAMP and glucocorticoid responsiveness of the rat carbamoylphosphate synthetase gene requires the interplay of upstream regulatory units</cite>. In: <cite style="font-style:italic">Biochimie</cite>. 89. Jahrgang, <span style="white-space:nowrap">Nr.<span style="display:inline-block;width:.2em"> </span>5</span>, Mai 2007, <span style="white-space:nowrap">S.<span style="display:inline-block;width:.2em"> </span>574–80</span>, <a href="/wiki/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.biochi.2006.12.013">10.1016/j.biochi.2006.12.013</a></span>, <a class="external mw-magiclink-pmid" rel="nofollow" href="//www.ncbi.nlm.nih.gov/pubmed/17397987?dopt=Abstract">PMID 17397987</a>.<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:Carbamoylphosphat-Synthetase+I&rft.atitle=cyclicAMP+and+glucocorticoid+responsiveness+of+the+rat+carbamoylphosphate+synthetase+gene+requires+the+interplay+of+upstream+regulatory+units&rft.au=Schoneveld+OJ%2C+Hoogenkamp+M%2C+Stallen+JM%2C+...&rft.date=2007-05&rft.doi=10.1016%2Fj.biochi.2006.12.013&rft.genre=journal&rft.issue=5&rft.jtitle=Biochimie&rft.pages=574-80&rft.pmid=17397987&rft.volume=89.+Jahrgang" style="display:none"> </span></span> </li> <li id="cite_note-5"><span class="mw-cite-backlink"><a href="#cite_ref-5">↑</a></span> <span class="reference-text">Butler SL, Dong H, Cardona D, <i>et al.</i>: <cite style="font-style:italic">The antigen for Hep Par 1 antibody is the urea cycle enzyme carbamoyl phosphate synthetase 1</cite>. In: <cite style="font-style:italic">Lab. Invest.</cite> 88. Jahrgang, <span style="white-space:nowrap">Nr.<span style="display:inline-block;width:.2em"> </span>1</span>, Januar 2008, <span style="white-space:nowrap">S.<span style="display:inline-block;width:.2em"> </span>78–88</span>, <a href="/wiki/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/labinvest.3700699">10.1038/labinvest.3700699</a></span>, <a class="external mw-magiclink-pmid" rel="nofollow" href="//www.ncbi.nlm.nih.gov/pubmed/18026163?dopt=Abstract">PMID 18026163</a>.<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:Carbamoylphosphat-Synthetase+I&rft.atitle=The+antigen+for+Hep+Par+1+antibody+is+the+urea+cycle+enzyme+carbamoyl+phosphate+synthetase+1&rft.au=Butler+SL%2C+Dong+H%2C+Cardona+D%2C+...&rft.date=2008-01&rft.doi=10.1038%2Flabinvest.3700699&rft.genre=journal&rft.issue=1&rft.jtitle=Lab.+Invest.&rft.pages=78-88&rft.pmid=18026163&rft.volume=88.+Jahrgang" style="display:none"> </span></span> </li> </ol></div><noscript><img src="https://login.wikimedia.org/wiki/Special:CentralAutoLogin/start?type=1x1&useformat=desktop" alt="" width="1" height="1" style="border: none; position: absolute;"></noscript> <div class="printfooter" data-nosnippet="">Abgerufen von „<a dir="ltr" href="https://de.wikipedia.org/w/index.php?title=Carbamoylphosphat-Synthetase_I&oldid=237009044">https://de.wikipedia.org/w/index.php?title=Carbamoylphosphat-Synthetase_I&oldid=237009044</a>“</div></div> <div id="catlinks" class="catlinks" data-mw="interface"><div id="mw-normal-catlinks" class="mw-normal-catlinks"><a href="/wiki/Wikipedia:Kategorien" title="Wikipedia:Kategorien">Kategorien</a>: <ul><li><a href="/wiki/Kategorie:Ligase" title="Kategorie:Ligase">Ligase</a></li><li><a href="/wiki/Kategorie:Codiert_auf_Chromosom_2_(Mensch)" title="Kategorie:Codiert auf Chromosom 2 (Mensch)">Codiert auf Chromosom 2 (Mensch)</a></li></ul></div><div id="mw-hidden-catlinks" class="mw-hidden-catlinks mw-hidden-cats-hidden">Versteckte Kategorien: <ul><li><a href="/wiki/Kategorie:Wikipedia:Vorlagenfehler/Vorlage:Cite_journal/tempor%C3%A4r" title="Kategorie:Wikipedia:Vorlagenfehler/Vorlage:Cite journal/temporär">Wikipedia:Vorlagenfehler/Vorlage:Cite journal/temporär</a></li><li><a href="/wiki/Kategorie:Wikipedia:Proteinbild_nicht_vorhanden" title="Kategorie:Wikipedia:Proteinbild nicht vorhanden">Wikipedia:Proteinbild nicht vorhanden</a></li></ul></div></div> </div> </div> <div id="mw-navigation"> <h2>Navigationsmenü</h2> <div id="mw-head"> <nav id="p-personal" class="mw-portlet mw-portlet-personal vector-user-menu-legacy vector-menu" aria-labelledby="p-personal-label" > <h3 id="p-personal-label" class="vector-menu-heading " > <span class="vector-menu-heading-label">Meine Werkzeuge</span> </h3> <div class="vector-menu-content"> <ul class="vector-menu-content-list"> <li id="pt-anonuserpage" class="mw-list-item"><span title="Benutzerseite der IP-Adresse, von der aus du Änderungen durchführst">Nicht angemeldet</span></li><li id="pt-anontalk" class="mw-list-item"><a href="/wiki/Spezial:Meine_Diskussionsseite" title="Diskussion über Änderungen von dieser IP-Adresse [n]" accesskey="n"><span>Diskussionsseite</span></a></li><li id="pt-anoncontribs" class="mw-list-item"><a href="/wiki/Spezial:Meine_Beitr%C3%A4ge" title="Eine Liste der Bearbeitungen, die von dieser IP-Adresse gemacht wurden [y]" accesskey="y"><span>Beiträge</span></a></li><li id="pt-createaccount" class="mw-list-item"><a href="/w/index.php?title=Spezial:Benutzerkonto_anlegen&returnto=Carbamoylphosphat-Synthetase+I" title="Wir ermutigen dich dazu, ein Benutzerkonto zu erstellen und dich anzumelden. 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