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	<title>精氨基琥珀酸合酶 - 版本历史</title>
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	<updated>2026-04-20T18:10:24Z</updated>
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		<title>123.130.221.191：建立内容为“{{enzyme | Name = 精氨基琥珀酸合酶 | EC_number = 6.3.4.5 | CAS_number = 9023-58-9 | IUBMB_EC_number = 6/3/4/5 | GO_code = 0004055 | image = Protein_ASS1_P…”的新页面</title>
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		<updated>2021-07-21T19:11:26Z</updated>

		<summary type="html">&lt;p&gt;建立内容为“{{enzyme | Name = 精氨基琥珀酸合酶 | EC_number = 6.3.4.5 | CAS_number = 9023-58-9 | IUBMB_EC_number = 6/3/4/5 | GO_code = 0004055 | image = Protein_ASS1_P…”的新页面&lt;/p&gt;
&lt;p&gt;&lt;b&gt;新页面&lt;/b&gt;&lt;/p&gt;&lt;div&gt;{{enzyme&lt;br /&gt;
| Name = 精氨基琥珀酸合酶&lt;br /&gt;
| EC_number = 6.3.4.5&lt;br /&gt;
| CAS_number = 9023-58-9&lt;br /&gt;
| IUBMB_EC_number = 6/3/4/5&lt;br /&gt;
| GO_code = 0004055&lt;br /&gt;
| image = Protein_ASS1_PDB_2nz2.png&lt;br /&gt;
| width = &lt;br /&gt;
| caption =  Crystallographic structure of human argininosuccinate synthetase.&amp;lt;ref name=&amp;quot;pmid18323623&amp;quot;&amp;gt;{{PDB|2nz2}}; {{cite journal | author = Karlberg T, Collins R, van den Berg S, Flores A, Hammarström M, Högbom M, Holmberg Schiavone L, Uppenberg J | title = Structure of human argininosuccinate synthetase | journal = Acta Crystallogr. D Biol. Crystallogr. | volume = 64 | issue = Pt 3 | pages = 279–86 | year = 2008 | month = March | pmid = 18323623 | doi = 10.1107/S0907444907067455 | url = | issn = }}&amp;lt;/ref&amp;gt;&lt;br /&gt;
}}&lt;br /&gt;
&lt;br /&gt;
{{Protein&lt;br /&gt;
   |Name=[[精氨基琥珀酸合酶1]]&lt;br /&gt;
   |image=&lt;br /&gt;
   |caption=&lt;br /&gt;
   |Symbol=[[精氨基琥珀酸合酶1|ASS1]]&lt;br /&gt;
   |AltSymbols=&lt;br /&gt;
   |HGNCid=758&lt;br /&gt;
   |Chromosome=9&lt;br /&gt;
   |Arm=q&lt;br /&gt;
   |Band=34.1&lt;br /&gt;
   |LocusSupplementaryData=&lt;br /&gt;
   |ECnumber=6.3.4.5&lt;br /&gt;
   |OMIM=603470&lt;br /&gt;
   |EntrezGene=445&lt;br /&gt;
   |RefSeq=NM_000050&lt;br /&gt;
   |UniProt=P00966&lt;br /&gt;
   |PDB=&lt;br /&gt;
}}&lt;br /&gt;
&lt;br /&gt;
{{Infobox protein family&lt;br /&gt;
| Symbol = Arginosuc_synth&lt;br /&gt;
| Name = 精氨基琥珀酸合酶&lt;br /&gt;
| image = PDB 1j21 EBI.jpg&lt;br /&gt;
| width = &lt;br /&gt;
| caption = crystal structure of thermus thermophilus hb8 argininosuccinate synthetase in complex with atp and citrulline&lt;br /&gt;
| Pfam = PF00764&lt;br /&gt;
| Pfam_clan = CL0039 &lt;br /&gt;
| InterPro = IPR001518&lt;br /&gt;
| SMART = &lt;br /&gt;
| PROSITE = PDOC00488&lt;br /&gt;
| MEROPS = &lt;br /&gt;
| SCOP = 1kp2&lt;br /&gt;
| TCDB = &lt;br /&gt;
| OPM family = &lt;br /&gt;
| OPM protein = &lt;br /&gt;
| CAZy = &lt;br /&gt;
| CDD = &lt;br /&gt;
}}&lt;br /&gt;
'''精氨基琥珀酸合酶'''（{{lang-en|Argininosuccinate synthase}}）或称'''精氨[[琥珀酸]][[合酶]]'''是一种从[[瓜氨酸]]及[[天冬氨酸]][[催化]]合成[[精氨基琥珀酸]]的酶（[[EC编号|EC]]6.3.4.5）。它是负责[[尿素循环]]中的第3个步骤，及瓜氨酸与[[一氧化氮]]循环其中的一个反应。&lt;br /&gt;
&lt;br /&gt;
== [[基因]]及[[病理]] ==&lt;br /&gt;
&lt;br /&gt;
为精氨基琥珀酸合酶编码的基因，是位于第9号的[[染色体]]，称为'''精氨基琥珀酸合成基因'''。在人类，这种基因主要在[[肝脏]]及[[肾脏]]的[[细胞]]内。&lt;br /&gt;
&lt;br /&gt;
精氨基琥珀酸合成基因的[[突变]]，有超过50种是会导致[[瓜氨酸血症|瓜氨酸血症1型]]。大部份是将合酶内的[[胺基酸]]取代成另一种，这会影响酶本身的结构，及它与瓜氨酸、天冬氨酸及其他[[分子]]的结合。小部份会引至在[[尿素]]循[[环中]]分泌不正常的酶而未能发挥它应有的功效。&lt;br /&gt;
&lt;br /&gt;
基因的缺憾亦会破坏尿素循环的第3步骤，阻碍肝脏将多余的氮转化为尿素。这会导致[[血液]]内的氮（以氨的形式）及其他尿素循环的副产物（如瓜氨酸）的上升。氨是有毒物质，影响[[神经系统]]，过多的积聚会引起[[食欲不振]]、[[呕吐]]、[[癫痫]]及其他[[瓜氨酸血症]]1型征状。&lt;br /&gt;
&lt;br /&gt;
==参考文献==&lt;br /&gt;
&lt;br /&gt;
{{reflist}}&lt;br /&gt;
{{尿素循环酶类}}&lt;br /&gt;
{{碳-碳连接酶}}&lt;br /&gt;
[[Category:酶]] [[分类:尿素循环]]&lt;br /&gt;
==参考来源==&lt;br /&gt;
*[http://zh.wikipedia.org/wiki/%E7%B2%BE%E6%B0%A8%E5%9F%BA%E7%90%A5%E7%8F%80%E9%85%B8%E5%90%88%E9%85%B6 维基百科-精氨基琥珀酸合酶]&lt;/div&gt;</summary>
		<author><name>123.130.221.191</name></author>
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