Argininosuccinic acid explained

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.

Synonyms

Argininosuccinate[4]

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 . 10.1007/8904_2016_2 . 2192-8304 . 5509549 . 27515243. 978-3-662-55585-9 .
  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 . 10.1007/BF02312521 . 0141-8955 . 6777600. 27912636 .
  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 . 10.1006/bbrc.1997.6854 . 0006-291X . 9207208.
  4. Web site: PubChem . Argininosuccinate . 2023-01-01 . pubchem.ncbi.nlm.nih.gov . en.

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