Argininosuccinic acid

Argininosuccinic acid
Names
	IUPAC name N-[{[(4S)-4-amino-4-carboxybutyl]amino}(imino)methyl]-L-aspartic acid
Identifiers
CAS Number	2387-71-5 ;
3D model (JSmol)	Interactive image;
ChemSpider	16059 ;
IUPHAR/BPS	5324;
PubChem CID	16950;
UNII	T67NZ5MU4H ;
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 Key: KDZOASGQNOPSCU-WDSKDSINSA-N ; 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 their standard state (at 25 °C [77 °F], 100 kPa). verify (what is ?) Infobox references

Argininosuccinic acid is a non-proteinogenic amino acid that is an important intermediate in the urea cycle. ^[1] It is also known as argininosuccinate.^[2]

Reactions

Some cells synthesize argininosuccinic acid from citrulline and aspartic acid and use it as a precursor for arginine in the urea cycle (or citrulline-NO cycle), releasing fumarate as a by-product to enter the TCA cycle. The enzyme that catalyzes the reaction is argininosuccinate synthetase.^[3]^[4]

Argininosuccinic acid is a precursor to fumarate in the citric acid cycle via argininosuccinate 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. Retrieved 2023-01-01.
^ 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, retrieved 2023-01-01