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Argininosuccinic acid

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Argininosuccinic acid
Names

IUPAC name N-[{[(4S)-4-amino-4-carboxybutyl]amino}(imino)methyl]-L-aspartic acid
Identifiers
CAS Number	2387-71-5^N
3D model (JSmol)	Interactive image
ChemSpider	16059^Y
IUPHAR/BPS	5324
PubChem CID	16950
UNII	T67NZ5MU4H^Y
CompTox Dashboard(EPA)	DTXSID80178574
InChI InChI=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^Y Key: KDZOASGQNOPSCU-WDSKDSINSA-N^Y 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 Key: KDZOASGQNOPSCU-WDSKDSINBJ
SMILES O=C(O)C[C@@H](C(=O)O)NC(=N/CCC[C@H](N)C(=O)O)/N
Properties
Chemical formula	C₁₀H₁₈N₄O₆
Molar mass	290.27312
Except where otherwise noted, data are given for materials in theirstandard state (at 25 °C [77 °F], 100 kPa). N verify (what is ^YN ?) Infobox references

Chemical compound

Argininosuccinic acid is anon-proteinogenic amino acid that is an important intermediate in theurea cycle.^[1] It is also known asargininosuccinate.^[2]

Some cells synthesize argininosuccinic acid fromcitrulline andaspartic acid and use it as a precursor forarginine in theurea cycle (orcitrulline-NO cycle), releasingfumarate as a by-product to enter theTCA cycle. The enzyme that catalyzes the reaction isargininosuccinate synthetase.^[3]^[4]

Argininosuccinic acid is a precursor tofumarate in thecitric acid cycle viaargininosuccinate lyase.^[5]

^Ganetzky, Rebecca D.; Bedoukian, Emma; Deardorff, Matthew A.; Ficicioglu, Can (2017)."Argininosuccinic Acid Lyase Deficiency Missed by Newborn Screen".JIMD Reports.34:43–47.doi:10.1007/8904_2016_2.ISBN 978-3-662-55585-9.ISSN 2192-8304.PMC 5509549.PMID 27515243.
^PubChem."Argininosuccinate".pubchem.ncbi.nlm.nih.gov. Retrieved2023-01-01.
^González-Noriega, A.; Verduzco, J.; Prieto, E.; Velázquez, A. (1980). "Argininosuccinic acid synthetase deficiency in a hamster cell line and its complementation of argininosuccinic aciduria human fibroblasts".Journal of Inherited Metabolic Disease.3 (2):45–48.doi:10.1007/BF02312521.ISSN 0141-8955.PMID 6777600.S2CID 27912636.
^Nakata, M.; Yada, T.; Nakagawa, S.; Kobayashi, K.; Maruyama, I. (1997-06-27). "Citrulline-argininosuccinate-arginine cycle coupled to Ca2+-signaling in rat pancreatic beta-cells".Biochemical and Biophysical Research Communications.235 (3):619–624.doi:10.1006/bbrc.1997.6854.ISSN 0006-291X.PMID 9207208.
^Nagamani, Sandesh C. Sreenath; Erez, Ayelet; Lee, Brendan (1993), Adam, Margaret P.; Everman, David B.; Mirzaa, Ghayda M.; Pagon, Roberta A. (eds.),"Argininosuccinate Lyase Deficiency",GeneReviews®, Seattle (WA): University of Washington, Seattle,PMID 21290785, retrieved2023-01-01

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