Chemistry:Argininosuccinic acid

From HandWiki
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
UNII
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).
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Infobox references
Tracking categories (test):

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



  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. PMID 27515243. 
  2. 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. https://pubmed.ncbi.nlm.nih.gov/6777600/. 
  3. 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. https://pubmed.ncbi.nlm.nih.gov/9207208/. 
  4. Nagamani, Sandesh C. Sreenath; Erez, Ayelet; Lee, Brendan (1993), Adam, Margaret P.; Everman, David B.; Mirzaa, Ghayda M. et al., eds., "Argininosuccinate Lyase Deficiency", GeneReviews® (Seattle (WA): University of Washington, Seattle), PMID 21290785, http://www.ncbi.nlm.nih.gov/books/NBK51784/, retrieved 2023-01-01 
  5. PubChem. "Argininosuccinate" (in en). https://pubchem.ncbi.nlm.nih.gov/compound/828.