We are currently updating the database - data may be missing for the next 10 minutes. We apologize for any inconvenience.

Bovine Metabolome Database



Showing metabocard for Argininosuccinic acid (BMDB00052)

Legend: metabolite field enzyme field

Version 1.0
Creation Date 2005-11-16 15:48:42
Update Date 2009-07-27 11:46:04
Accession Number BMDB00052
Common Name Argininosuccinic acid
Description Arginosuccinic acid is a basic amino acid. Some cells synthesize it from citrulline, 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. Argininosuccinic acid is a precursor to fumarate in the citric acid cycle via argininosuccinate lyase. Defects in the arginosuccinate lyase enzyme can lead to arginosuccinate lyase deficiency. Argininosuccinate (ASA) lyase deficiency results in defective cleavage of ASA. This leads to an accumulation of ASA in cells and an excessive excretion of ASA in urine (arginosuccinic aciduria). In virtually all respects, this disorder shares the characteristics of other urea cycle defects. The most important characteristic of ASA lyase deficiency is its propensity to cause hyperammonemia in affected individuals. ASA in affected individuals is excreted by the kidney at a rate practically equivalent to the glomerular filtration rate (GFR). Whether ASA itself causes a degree of toxicity due to hepatocellular accumulation is unknown; such an effect could help explain hyperammonemia development in affected individuals. Regardless, the name of the disease is derived from the rapid clearance of ASA in urine, although elevated levels of ASA can be found in plasma. ASA lyase deficiency is associated with high mortality and morbidity rates. Symptoms of ASA lyase deficiency include anorexia, irritability rapid breathing, lethargy and vomiting. Extreme symptoms include coma and cerebral edema.
Synonyms
  1. Argininosuccinate
  2. Argininosuccinic acid
  3. Arginosuccinate
  4. Arginosuccinic acid
  5. L-Argininosuccinate
  6. L-Argininosuccinic acid
  7. L-Arginosuccinate
  8. L-Arginosuccinic acid
  9. N-(((4-amino-4-carboxybutyl)amino)iminomethyl)-L-Aspartate
  10. N-(((4-amino-4-carboxybutyl)amino)iminomethyl)-L-Aspartic acid
  11. N-[(4-amino-4-carboxybutyl)amidino]-L-Aspartate
  12. N-[(4-amino-4-carboxybutyl)amidino]-L-Aspartic acid
  13. N-[[(4-amino-4-carboxybutyl)amino]iminomethyl]-L-Aspartate
  14. N-[[(4-amino-4-carboxybutyl)amino]iminomethyl]-L-Aspartic acid
  15. 2-(N(omega)-L-arginino)succinate
  16. 2-(N(omega)-L-arginino)succinic acid
  17. N-(L-arginino) succinate
  18. N-(L-arginino) succinic acid
  19. 2-(Nw-L-arginino)butanedioic acid
  20. 2-(N(omega)-L-arginine)succinate
  21. 2-(N(omega)-L-arginine)succinic acid
  22. N-(L-Arginino)succinate
  23. N-(L-Arginino)succinic acid
  24. ASA
  25. N(omega)-(L-arginino)succinate
  26. N(omega)-(L-arginino)succinic acid
  27. 2-(Nw-L-arginino)butanedioate
Chemical IUPAC Name 2-[amino-(4-amino-4-carboxy-butyl)imino-methyl]aminobutanedioic acid
Chemical Formula C10H18N4O6
Chemical Structure Structure
Chemical Taxonomy
Kingdom
  • Organic
Super Class
  • Amino acids and Amino Acid conjugates
Class
  • Amino Acids
Sub Class
  • NA
Family
  • Mammalian_Metabolite
Species
  • primary amine; primary aliphatic amine (alkylamine); carboxylic acid; guanidine; alpha-aminoacid
Biofunction
  • Protein synthesis, amino acid biosynthesis
Application
Source
  • Endogenous
Average Molecular Weight 290.273
Monoisotopic Molecular Weight 290.122620
Isomeric SMILES N[C@@H](CCCNN=CN[C@@H](CC(O)=O)C(O)=O)C(O)=O
Canonical SMILES NC(CCCNN=CNC(CC(O)=O)C(O)=O)C(O)=O
KEGG Compound ID C03406 Link Image
BioCyc ID Not Available
BiGG ID 41790 Link Image
Wikipedia Link Argininosuccinic acid Link Image
METLIN ID 5115 Link Image
PubChem Compound 439998 Link Image
PubChem Substance 8144809 Link Image
ChEBI ID 15682 Link Image
CAS Registry Number 2387-71-5
InChI Identifier InChI=1/C10H18N4O6/c11-6(9(17)18)2-1-3-13-14-5-12-7(10(19)20)4-8(15)16/h5-7,13H,1-4,11H2,(H,12,14)(H,15,16)(H,17,18)(H,19,20)/t6-,7-/m0/s1
Synthesis Reference Hagino, Koji; Nakanishi, Toshihide. Fermentative production of L-arginosuccinic acid. Jpn. Kokai Tokkyo Koho (1980), 3 pp.
Melting Point (Experimental) Not Available
Experimental Water Solubility Not Available Source: PhysProp
Predicted Water Solubility 0.853 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS
Physiological Charge -1
State Solid
Experimental LogP/Hydrophobicity Not Available Source: PhysProp
Predicted LogP/Hydrophobicity -2.28 [Predicted by ALOGPS]; -6.3 [Predicted by PubChem via XLOGP] Calculated using ALOGPS
Material Safety Data Sheet (MSDS)
MOL File Show
SDF File Show
PDB File Show
2D Structure
3D Structure
Experimental PDB ID Not Available
Experimental 1H NMR Spectrum Download Spectrum
Download FID (Bruker)
Show Experimental Conditions Link Image
Experimental 13C NMR Spectrum Not Available
Experimental 13C HSQC Spectrum Download Spectrum
Download FID (Bruker)
Show Experimental Conditions Link Image
Predicted 1H NMR Spectrum Show Image
Show Peaklist
Predicted 13C NMR Spectrum Show Image
Show Peaklist
Mass Spectrum Not Available
Simplified TOCSY Spectrum Not Available
BMRB Spectrum Not Available
Cellular Location
  • Cytoplasm
Biofluid Location Not Available
Tissue Location
Tissue References
Kidney
Concentrations (Normal) Not Available
Concentrations (Abnormal) Not Available
Pathway Names
  • Alanine and aspartate metabolism
  • Arginine and Proline Metabolism
  • Urea Cycle and Metabolism of Amino Groups
HMDB Pathways
Name Alanine and aspartate metabolism
Image Show Link Image
Name Arginine and Proline Metabolism
Image Show Link Image
Name Urea Cycle and Metabolism of Amino Groups
Image Show Link Image
KEGG Pathways
Name Alanine and aspartate metabolism
Image Show Link Image
Name Arginine and Proline Metabolism
Image Show Link Image
Name Urea Cycle and Metabolism of Amino Groups
Image Show Link Image
SimCell Pathways
Name Alanine and aspartate metabolism
Image Show Link Image
Graph Show Link Image
SBML Download (XML) Link Image
Name Arginine and Proline Metabolism
Image Show Link Image
Graph
SBML
Name Urea Cycle and Metabolism of Amino Groups
Image Show Link Image
Graph Show Link Image
SBML Download (XML) Link Image
General References
  1. Wikipedia Link Image
Metabolic Enzymes
  1. Argininosuccinate synthase
  2. Argininosuccinate lyase
Enzyme 1 [top]
Enzyme 1 ID 237
Enzyme 1 Name Argininosuccinate synthase
Enzyme 1 Synonyms
  1. Citrulline--aspartate ligase
Enzyme 1 Gene Name ASS1
Enzyme 1 Protein Sequence >Argininosuccinate synthase
MSGKGSVVLAYSGGLDTSCILVWLKEQGYDVIAYLANIGQKEDFEEARKKALKLGAKKVF
IEDISKEFVEEFIWPAIQSSALYEDRYLLGTSLARPCIARKQVEIAQREGAKYVSHGATG
KGNDQIRFELTCYSLAPQIKVIAPWRMPEFYNRFQGRNDLMEYAKQHGIPVPVTPKNPWS
MDENLMHISYEAGILENPKNQAPPGLYTKTQDPAKAPNSPDMLEIEFKKGVPVKVTNVGD
GTTHSTALELFLYLNEVAGKHGVGRIDIVENRFIGMKSRGIYETPAGTILYHAHLDIEAF
TMDREVRKIKQGLGLKFAELVYTGFWHSPECEFVRHCIAKSQERVEGKVQVSVFKGQVYI
LGRESPLSLYNEELVSMNVQGDYEPVDATGFININSLRLKEYHRLQNKVTAK
Enzyme 1 Number of Residues 412
Enzyme 1 Molecular Weight 46416.9
Enzyme 1 Theoretical pI 7.61
Enzyme 1 GO Classification
Function
Process
Component
Enzyme 1 General Function Nucleotide transport and metabolism
Enzyme 1 Specific Function ATP + L-citrulline + L-aspartate = AMP + diphosphate + N(omega)-(L-arginino)succinate
Enzyme 1 Pathways
  • Nitrogen metabolism
  • urea cycle
  • (N(omega)-L- arginino)succinate from L-aspartate and L-citrulline:step 1/1
Enzyme 1 Reactions Not Available
Enzyme 1 Pfam Domain Function
Enzyme 1 Signals
  • None
Enzyme 1 Transmembrane Regions
  • None
Enzyme 1 Essentiality Not Available
Enzyme 1 GenBank ID Protein 162697 Link Image
Enzyme 1 UniProtKB/Swiss-Prot ID P14568 Link Image
Enzyme 1 UniProtKB/Swiss-Prot Entry Name ASSY_BOVIN Link Image
Enzyme 1 PDB ID Not Available
Enzyme 1 Cellular Location Not Available
Enzyme 1 Gene Sequence >1239 bp
ATGTCCGGCAAAGGCTCCGTGGTTCTGGCCTACAGTGGGGGCCTGGACACCTCCTGCATC
CTCGTGTGGCTGAAGGAGCAAGGCTATGACGTCATTGCCTACCTGGCCAACATCGGCCAG
AAAGAAGACTTTGAGGAAGCCAGGAAGAAGGCGCTGAAGCTTGGGGCCAAAAAGGTGTTC
ATTGAGGACATCAGCAAGGAGTTTGTGGAGGAGTTCATCTGGCCGGCCATCCAGTCCAGC
GCACTGTACGAGGACCGATACCTCCTGGGCACCTCTCTCGCCAGGCCCTGCATCGCCCGC
AAGCAGGTGGAGATCGCCCAGCGAGAAGGAGCCAAGTATGTGTCTCACGGCGCCACAGGA
AAGGGGAACGACCAGATCCGGTTTGAGCTCACCTGCTACTCGCTGGCCCCACAGATCAAG
GTCATCGCTCCCTGGAGGATGCCCGAGTTCTATAACCGCTTCCAGGGCCGCAACGATCTG
ATGGAGTATGCGAAGCAACATGGAATCCCCGTCCCAGTCACCCCCAAGAACCCGTGGAGC
ATGGACGAGAACCTGATGCATATCAGCTACGAGGCTGGAATCCTGGAGAACCCCAAGAAC
CAAGCGCCTCCAGGCCTCTACACAAAGACCCAGGACCCGGCCAAAGCCCCCAACAGCCCG
GACATGCTCGAGATCGAGTTCAAGAAAGGGGTCCCCGTGAAGGTGACCAACGTCGGGGAT
GGCACCACCCACAGCACAGCGTTGGAGCTTTTCCTGTACCTGAATGAAGTCGCTGGCAAG
CACGGCGTGGGCCGCATCGACATCGTGGAAAACCGCTTCATCGGGATGAAGTCCCGGGGT
ATCTACGAGACCCCAGCGGGGACGATCCTTTACCACGCTCATTTAGACATCGAGGCCTTC
ACCATGGACCGGGAAGTGCGCAAAATCAAGCAAGGCCTCGGCTTGAAATTCGCCGAGCTG
GTCTACACGGGTTTCTGGCACAGCCCCGAGTGTGAATTTGTCCGCCACTGCATTGCCAAG
TCCCAGGAGCGCGTGGAAGGGAAAGTGCAGGTGTCCGTCTTCAAGGGCCAGGTGTACATC
CTTGGCCGGGAGTCCCCACTGTCCCTCTACAATGAGGAGCTCGTGAGCATGAACGTGCAG
GGAGACTACGAGCCGGTTGATGCCACTGGTTTCATCAACATCAATTCCCTCAGGCTGAAG
GAATATCATCGCCTCCAGAACAAGGTCACCGCCAAATAG
Enzyme 1 GenBank Gene ID M26198 Link Image
Enzyme 1 GeneCard ID ASS1 Link Image
Enzyme 1 GenAtlas ID Not Available
Enzyme 1 HGNC ID Not Available
Enzyme 1 Chromosome Location Chromosome:9
Enzyme 1 Locus 9q34.1
Enzyme 1 SNPs SNPJam Report Link Image
Enzyme 1 General References
  1. [PubMed Link Image]
Enzyme 1 Metabolite References Not Available
Enzyme 2 [top]
Enzyme 2 ID 238
Enzyme 2 Name Argininosuccinate lyase
Enzyme 2 Synonyms
  1. ASAL
  2. Arginosuccinase
Enzyme 2 Gene Name ASL
Enzyme 2 Protein Sequence >Argininosuccinate lyase
MASESGKLWGGRFVGTVDPIMEKFNSSITYDRHLWEADVQGSKAYSRGLEKAGLLTKAEM
DQILHGLDKVAEEWAQGTFKLNPNDEDIHTANERRLKELIGETAGKLHTGRSRNDQVVTD
LRLWMRQNCSMLSALLCELIRTMVDRAEAERDVLFPGYTHLQRAQPIRWSHWILSHAVAL
TRDSERLLEVRKRINVLPLGSGAIAGNPLGVDRELLRAELDFGAITLNSMDATSERDFVA
EFLFWASLCMTHLSRMAEDLILYGTKEFSFVQLSDAYSTGSSLMPQKKNPDSLELIRSKA
GRVFGRCAGLLMTLKGLPSTYNKDLQEDKEAVFEVSDTMSAVLQVATGVISTLQIHRENM
GRALSPDMLATDLAYYLVRKGMPFRQAHEASGKAVFMAETKGVALNQLSLQELQTISPLF
SGDVSHVWDYGHSVEQYEALGGTARSSVDWQIGQLRALLRAQQTESPPHASPK
Enzyme 2 Number of Residues 473
Enzyme 2 Molecular Weight 52742.6
Enzyme 2 Theoretical pI 6.46
Enzyme 2 GO Classification
Function
Process
Component
Enzyme 2 General Function Amino acid transport and metabolism
Enzyme 2 Specific Function 2-(N(omega)-L-arginino)succinate = fumarate + L-arginine
Enzyme 2 Pathways
  • Nitrogen metabolism
  • urea cycle
  • L-arginine and fumarate from (N(omega)-L-arginino)succinate:step 1/1
Enzyme 2 Reactions Not Available
Enzyme 2 Pfam Domain Function
Enzyme 2 Signals
  • None
Enzyme 2 Transmembrane Regions
  • None
Enzyme 2 Essentiality Not Available
Enzyme 2 GenBank ID Protein 74354042 Link Image
Enzyme 2 UniProtKB/Swiss-Prot ID Q3SZJ0 Link Image
Enzyme 2 UniProtKB/Swiss-Prot Entry Name ARLY_BOVIN Link Image
Enzyme 2 PDB ID 1K62 Link Image
Enzyme 2 PDB File Show
Enzyme 2 3D Structure
Enzyme 2 Cellular Location Not Available
Enzyme 2 Gene Sequence >1422 bp
ATGGCATCGGAGAGCGGGAAGCTATGGGGTGGCCGGTTTGTGGGCACAGTGGACCCCATC
ATGGAAAAGTTCAACTCGTCCATCACGTACGACCGGCACCTCTGGGAGGCGGACGTGCAG
GGCAGCAAAGCCTACAGCCGGGGCCTGGAGAAGGCGGGGCTCCTCACCAAAGCCGAGATG
GACCAGATACTCCATGGCCTGGACAAGGTAGCTGAGGAGTGGGCTCAGGGCACCTTCAAA
CTAAACCCCAATGATGAGGACATCCATACGGCCAATGAGCGGCGTCTGAAGGAGCTCATT
GGTGAAACCGCGGGGAAGCTGCACACGGGACGAAGTCGGAACGACCAGGTGGTCACTGAC
CTCAGGCTGTGGATGCGGCAGAATTGCTCCATGCTCTCAGCCCTCCTCTGCGAACTCATC
AGAACCATGGTGGATCGGGCAGAGGCGGAACGTGATGTCCTCTTCCCAGGGTACACACAC
CTGCAGAGGGCTCAGCCGATCCGCTGGAGCCACTGGATCCTGAGCCATGCGGTGGCTCTG
ACCAGAGACTCTGAAAGGCTGCTGGAGGTGCGGAAGCGGATCAACGTCCTGCCCCTGGGG
AGCGGGGCCATCGCAGGCAACCCCCTGGGTGTGGACCGGGAACTGCTCCGAGCAGAACTG
GACTTTGGGGCCATCACTCTCAACAGCATGGATGCCACCAGTGAGCGAGACTTTGTGGCC
GAGTTCCTATTCTGGGCTTCGCTGTGCATGACCCACCTCAGCAGGATGGCTGAGGATCTC
ATCCTCTATGGCACCAAGGAGTTCAGCTTCGTGCAGCTCTCAGATGCCTACAGCACCGGA
AGCAGTCTGATGCCCCAGAAGAAAAACCCAGACAGCCTGGAGCTGATCCGGAGCAAGGCG
GGGCGAGTGTTTGGGCGGTGTGCTGGGCTCCTGATGACACTCAAGGGACTTCCGAGCACC
TACAACAAGGACTTACAGGAAGACAAGGAAGCCGTGTTTGAAGTGTCCGACACCATGAGC
GCTGTCCTCCAAGTGGCCACTGGTGTCATCTCTACCCTGCAGATTCACCGTGAGAACATG
GGACGGGCTCTGAGTCCTGACATGCTAGCCACTGACCTCGCCTACTACCTGGTCCGAAAA
GGGATGCCATTCCGCCAGGCCCACGAGGCCTCCGGGAAAGCCGTGTTCATGGCTGAGACC
AAGGGGGTCGCCCTCAATCAGCTGTCACTGCAGGAGCTGCAGACCATCAGCCCCCTGTTC
TCGGGCGACGTGAGCCACGTGTGGGACTACGGACACAGCGTGGAGCAGTACGAGGCCCTG
GGAGGCACCGCGCGCTCCAGCGTTGACTGGCAGATCGGCCAGCTGCGAGCTCTCCTCCGG
GCACAGCAGACCGAGAGCCCTCCCCACGCCTCTCCCAAATAA
Enzyme 2 GenBank Gene ID BC102831 Link Image
Enzyme 2 GeneCard ID ASL Link Image
Enzyme 2 GenAtlas ID Not Available
Enzyme 2 HGNC ID Not Available
Enzyme 2 Chromosome Location Chromosome:7
Enzyme 2 Locus 7cen-q11.2
Enzyme 2 SNPs SNPJam Report Link Image
Enzyme 2 General References Not Available
Enzyme 2 Metabolite References Not Available