| Revision as of 16:25, 26 September 2024 editTNstingray (talk | contribs)Extended confirmed users, Pending changes reviewers5,018 edits specifying split to arginine and fumarateTag: Visual edit← Previous edit | Latest revision as of 05:52, 2 January 2025 edit undoArthurfragoso (talk | contribs)Extended confirmed users2,080 edits Fixes image in dark mode | ||
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| | ImageFile=Argininosuccinic acid.svg | | ImageFile=Argininosuccinic acid.svg | ||
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| | IUPACName=''N''-amino}(imino)methyl]-<small>L</small>-aspartic acid | | IUPACName=''N''-amino}(imino)methyl]-<small>L</small>-aspartic acid | ||
Latest revision as of 05:52, 2 January 2025
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| Names | |
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| IUPAC name N-amino}(imino)methyl]-L-aspartic acid | |
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| 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
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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
- 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.
- PubChem. "Argininosuccinate". pubchem.ncbi.nlm.nih.gov. Retrieved 2023-01-01.
- 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
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