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Argininosuccinic acid

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Argininosuccinic acid
Names
IUPAC name N-amino}(imino)methyl]-L-aspartic acid
Identifiers
CAS Number
3D model (JSmol)
ChemSpider
IUPHAR/BPS
PubChem CID
UNII
CompTox Dashboard (EPA)
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/s1Key: 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/s1Key: KDZOASGQNOPSCU-WDSKDSINBJ
SMILES
  • O=C(O)C(C(=O)O)NC(=N/CCC(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 , 100 kPa). ☒N verify (what is  ?) Infobox references
Chemical compound

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

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.

Argininosuccinic acid is a precursor to fumarate in the citric acid cycle via argininosuccinate lyase.

See also

References

  1. 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.
  2. PubChem. "Argininosuccinate". pubchem.ncbi.nlm.nih.gov. Retrieved 2023-01-01.
  3. 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.
  4. 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.
  5. 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
Urea cycle Metabolic Pathway

Carbamoyl phosphate

L-citrulline
Pi

L-ornithine

Urea

L-aspartate

+ ATP
PPi + AMP H2O

L-argininosuccinate

L-arginine

Fumarate

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