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{{Unreferenced|date=December 2009}} |
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{{Chembox |
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{{Chembox |
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|ImageFile=Argininosuccinic acid.svg |
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|ImageSize=200px |
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| verifiedrevid = 457813295 |
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|IUPACName=''N''-amino}(imino)methyl]-<small>L</small>-aspartic acid |
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| ImageFile=Argininosuccinic acid.svg |
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|OtherNames= |
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| ImageClass = skin-invert |
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|Section1= {{Chembox Identifiers |
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| ImageSize=200px |
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| ChemSpiderID = 16059 |
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| IUPACName=''N''-amino}(imino)methyl]-<small>L</small>-aspartic acid |
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| OtherNames= |
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|Section1={{Chembox Identifiers |
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| IUPHAR_ligand = 5324 |
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| ChemSpiderID_Ref = {{chemspidercite|correct|chemspider}} |
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| ChemSpiderID = 16059 |
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| InChI = 1/C10H18N4O6/c11-5(8(17)18)2-1-3-13-10(12)14-6(9(19)20)4-7(15)16/h5-6H,1-4,11H2,(H,15,16)(H,17,18)(H,19,20)(H3,12,13,14)/t5-,6-/m0/s1 |
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| InChI = 1/C10H18N4O6/c11-5(8(17)18)2-1-3-13-10(12)14-6(9(19)20)4-7(15)16/h5-6H,1-4,11H2,(H,15,16)(H,17,18)(H,19,20)(H3,12,13,14)/t5-,6-/m0/s1 |
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| InChIKey = KDZOASGQNOPSCU-WDSKDSINBJ |
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| InChIKey = KDZOASGQNOPSCU-WDSKDSINBJ |
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| CASNo_Ref = {{cascite|changed|??}} |
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| CASNo = <!-- blanked - oldvalue: 2387-71-5 --> |
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| CASNo=2387-71-5 |
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| StdInChI = 1S/C10H18N4O6/c11-5(8(17)18)2-1-3-13-10(12)14-6(9(19)20)4-7(15)16/h5-6H,1-4,11H2,(H,15,16)(H,17,18)(H,19,20)(H3,12,13,14)/t5-,6-/m0/s1 |
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| UNII_Ref = {{fdacite|correct|FDA}} |
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| UNII = T67NZ5MU4H |
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| StdInChI_Ref = {{stdinchicite|correct|chemspider}} |
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| StdInChI = 1S/C10H18N4O6/c11-5(8(17)18)2-1-3-13-10(12)14-6(9(19)20)4-7(15)16/h5-6H,1-4,11H2,(H,15,16)(H,17,18)(H,19,20)(H3,12,13,14)/t5-,6-/m0/s1 |
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| StdInChIKey_Ref = {{stdinchicite|correct|chemspider}} |
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| StdInChIKey = KDZOASGQNOPSCU-WDSKDSINSA-N |
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| StdInChIKey = KDZOASGQNOPSCU-WDSKDSINSA-N |
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| PubChem=16950 |
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| PubChem=16950 |
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| SMILES = O=C(O)C(C(=O)O)NC(=N/CCC(N)C(=O)O)/N |
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| SMILES = O=C(O)C(C(=O)O)NC(=N/CCC(N)C(=O)O)/N |
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|Section2= {{Chembox Properties |
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|Section2={{Chembox Properties |
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| C=10|H=18|N=4|O=6 |
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| C=10 | H=18 | N=4 | O=6 |
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| MolarMass=290.27312 |
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| MolarMass=290.27312 |
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|Section3= {{Chembox Hazards |
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|Section3={{Chembox Hazards |
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'''Argininosuccinic acid''' is a ] that is an important intermediate in the ]. <ref>{{Cite journal |last1=Ganetzky |first1=Rebecca D. |last2=Bedoukian |first2=Emma |last3=Deardorff |first3=Matthew A. |last4=Ficicioglu |first4=Can |date=2017 |title=Argininosuccinic Acid Lyase Deficiency Missed by Newborn Screen |journal=JIMD Reports |volume=34 |pages=43–47 |doi=10.1007/8904_2016_2 |issn=2192-8304 |pmc=5509549 |pmid=27515243|isbn=978-3-662-55585-9 }}</ref> It is also known as '''argininosuccinate'''.<ref>{{Cite web |last=PubChem |title=Argininosuccinate |url=https://pubchem.ncbi.nlm.nih.gov/compound/828 |access-date=2023-01-01 |website=pubchem.ncbi.nlm.nih.gov |language=en}}</ref> |
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'''Argininosuccinic acid''' is a ] that is a ] ]. |
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==Reactions== |
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==Reactions== |
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Some cells synthesize argininosuccinic acid from ] and ] and use it as a precursor for ] in the ] (or ]), releasing ] as a by-product to enter the ]. The enzyme that catalyzes the reaction is ].<ref>{{Cite journal |last1=González-Noriega |first1=A. |last2=Verduzco |first2=J. |last3=Prieto |first3=E. |last4=Velázquez |first4=A. |date=1980 |title=Argininosuccinic acid synthetase deficiency in a hamster cell line and its complementation of argininosuccinic aciduria human fibroblasts |url=https://pubmed.ncbi.nlm.nih.gov/6777600/ |journal=Journal of Inherited Metabolic Disease |volume=3 |issue=2 |pages=45–48 |doi=10.1007/BF02312521 |issn=0141-8955 |pmid=6777600|s2cid=27912636 }}</ref><ref>{{Cite journal |last1=Nakata |first1=M. |last2=Yada |first2=T. |last3=Nakagawa |first3=S. |last4=Kobayashi |first4=K. |last5=Maruyama |first5=I. |date=1997-06-27 |title=Citrulline-argininosuccinate-arginine cycle coupled to Ca2+-signaling in rat pancreatic beta-cells |url=https://pubmed.ncbi.nlm.nih.gov/9207208/ |journal=Biochemical and Biophysical Research Communications |volume=235 |issue=3 |pages=619–624 |doi=10.1006/bbrc.1997.6854 |issn=0006-291X |pmid=9207208}}</ref> |
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Some cells synthesize argininosuccinic acid from ] and ] and use it as a precursor for ] in the ] or ]. The enzyme that catalyzes the reaction is ]. |
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Argininosuccinic acid is a precursor to ] in the ] via ].<ref>{{Citation |last1=Nagamani |first1=Sandesh C. Sreenath |title=Argininosuccinate Lyase Deficiency |date=1993 |url=http://www.ncbi.nlm.nih.gov/books/NBK51784/ |work=GeneReviews® |editor-last=Adam |editor-first=Margaret P. |place=Seattle (WA) |publisher=University of Washington, Seattle |pmid=21290785 |access-date=2023-01-01 |last2=Erez |first2=Ayelet |last3=Lee |first3=Brendan |editor2-last=Everman |editor2-first=David B. |editor3-last=Mirzaa |editor3-first=Ghayda M. |editor4-last=Pagon |editor4-first=Roberta A.}}</ref> |
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Argininosuccinic acid is a precursor to ] in the ] via ]. |
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==See also== |
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==See also== |
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* ] |
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== References == |
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{{Urea cycle}} |
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<references />{{Urea cycle}} |
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{{DEFAULTSORT:Argininosuccinic Acid}} |
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{{DEFAULTSORT:Argininosuccinic Acid}} |
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