From Wikipedia, the free encyclopedia
Argininosuccinic acid
Names
IUPAC name
N -[{[(4S )-4-amino-4-carboxybutyl]amino}(imino)methyl]-L -aspartic acid
Identifiers
ChemSpider
UNII
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
O=C(O)C[C@@H](C(=O)O)NC(=N/CCC[C@H](N)C(=O)O)/N
Properties
C 10 H 18 N 4 O 6
Molar mass
290.27312
Except where otherwise noted, data are given for materials in their
standard state (at 25 °C [77 °F], 100 kPa).
Chemical compound
Argininosuccinic acid is a
non-proteinogenic amino acid that is an important intermediate in the
urea cycle .
[1]
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 . The enzyme that catalyzes the reaction is
argininosuccinate synthetase .
[2]
[3]
Argininosuccinic acid is a precursor to
fumarate in the
citric acid cycle via
argininosuccinate lyase .
[4]
Synonyms
Argininosuccinate
[5]
See also
References
^ 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 .
^ 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
^ PubChem.
"Argininosuccinate" . pubchem.ncbi.nlm.nih.gov . Retrieved 2023-01-01 .