Argininosuccinic acid

Last updated
Argininosuccinic acid
Argininosuccinic acid.svg
Names
IUPAC name
N-[{[(4S)-4-amino-4-carboxybutyl]amino}(imino)methyl]-L-aspartic acid
Identifiers
3D model (JSmol)
ChemSpider
PubChem CID
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 Yes check.svgY
    Key: KDZOASGQNOPSCU-WDSKDSINSA-N Yes check.svgY
  • 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
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).
X mark.svgN  verify  (what is  Yes check.svgYX mark.svgN ?)

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]

Contents

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]

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
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