MiMeDB: Showing metabocard for Argininosuccinic acid (MMDBc0000327)

Record Information

Version

1.0

Status

Detected and Quantified

Creation Date

2020-12-10 18:28:48 UTC

Update Date

2024-04-30 19:31:47 UTC

Metabolite ID

MMDBc0000327

Metabolite Identification

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 argininosuccinate lyase enzyme can lead to argininosuccinate lyase deficiency, which is an inborn error of metabolism. 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 (argininosuccinic 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.

Structure

MOL 3D MOL SDF 3D SDF PDB 3D PDB SMILES InChI

HMDB0000052
     RDKit          3D
Argininosuccinic acid
 38 37  0  0  0  0  0  0  0  0999 V2000
   -1.7537    1.1031    1.3975 N   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2635    0.1775    0.6303 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.0404    0.4135    0.1178 N   0  0  0  0  0  0  0  0  0  0  0  0
    1.1901   -0.2059    0.7203 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.4371    0.2245   -0.0285 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.6871   -0.3917    0.5559 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.8487    0.1301   -0.2961 C   0  0  2  0  0  0  0  0  0  0  0  0
    4.8279    1.5801   -0.1710 N   0  0  0  0  0  0  0  0  0  0  0  0
    4.6665   -0.2449   -1.7043 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.5216    0.6172   -2.6111 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.6470   -1.5800   -2.0915 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9982   -0.9834    0.3383 N   0  0  0  0  0  0  0  0  0  0  0  0
   -3.3774   -0.9865   -0.1533 C   0  0  1  0  0  0  0  0  0  0  0  0
   -4.2994   -0.3156    0.7965 C   0  0  0  0  0  0  0  0  0  0  0  0
   -5.6851   -0.3741    0.3185 C   0  0  0  0  0  0  0  0  0  0  0  0
   -6.0243   -1.0415   -0.6769 O   0  0  0  0  0  0  0  0  0  0  0  0
   -6.6664    0.3593    1.0221 O   0  0  0  0  0  0  0  0  0  0  0  0
   -3.3269   -0.2232   -1.4294 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2197    0.2718   -1.8493 O   0  0  0  0  0  0  0  0  0  0  0  0
   -4.4053    0.0151   -2.2435 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2599    1.9719    1.6448 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.1488    1.0446   -0.7029 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.3115    0.0766    1.7748 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.0887   -1.3229    0.6093 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.5294    1.3397    0.0610 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.3588   -0.0287   -1.0867 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.8150   -0.0578    1.5964 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.6341   -1.4934    0.4785 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.7924   -0.2551    0.1438 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.6312    1.9888   -0.7046 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.8561    1.8742    0.8224 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.4503   -2.0237   -2.5060 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5714   -1.9222    0.4668 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.6908   -2.0048   -0.3675 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.2551   -0.7723    1.8256 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.0580    0.7674    0.9202 H   0  0  0  0  0  0  0  0  0  0  0  0
   -6.7943    1.3501    0.8576 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.8335    0.9221   -2.3876 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  2  3
  2  3  1  0
  3  4  1  0
  4  5  1  0
  5  6  1  0
  6  7  1  0
  7  8  1  0
  7  9  1  0
  9 10  2  0
  9 11  1  0
  2 12  1  0
 12 13  1  0
 13 14  1  0
 14 15  1  0
 15 16  2  0
 15 17  1  0
 13 18  1  0
 18 19  2  0
 18 20  1  0
  1 21  1  0
  3 22  1  0
  4 23  1  0
  4 24  1  0
  5 25  1  0
  5 26  1  0
  6 27  1  0
  6 28  1  0
  7 29  1  1
  8 30  1  0
  8 31  1  0
 11 32  1  0
 12 33  1  0
 13 34  1  6
 14 35  1  0
 14 36  1  0
 17 37  1  0
 20 38  1  0
M  END


  Mrv1652307082004522D          

 20 19  0  0  1  0            999 V2000
   -3.5735    0.0000    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8591    0.4124    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1446    0.0000    0.0000 C   0  0  2  0  0  0  0  0  0  0  0  0
   -1.4301    0.4124    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7156    0.0000    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0011    0.4124    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
    0.7132    0.0000    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1446   -0.8250    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7156   -0.8250    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
    1.4277    0.4124    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.1422    0.0000    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.8567    0.4124    0.0000 C   0  0  1  0  0  0  0  0  0  0  0  0
    3.5712    0.0000    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.8567    1.2374    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4301   -1.2375    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8591   -1.2375    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -4.2855    0.4167    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -3.5760   -0.8237    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.5714   -0.8251    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.2855    0.4126    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  2  1  1  0  0  0  0
  3  2  1  0  0  0  0
  4  3  1  0  0  0  0
  5  4  1  0  0  0  0
  6  5  1  0  0  0  0
  7  6  1  0  0  0  0
  3  8  1  6  0  0  0
  5  9  2  0  0  0  0
  7 10  1  0  0  0  0
 10 11  1  0  0  0  0
 12 13  1  0  0  0  0
 12 14  1  6  0  0  0
 11 12  1  0  0  0  0
 15  8  2  0  0  0  0
 16  8  1  0  0  0  0
 17  1  2  0  0  0  0
 18  1  1  0  0  0  0
 19 13  2  0  0  0  0
 20 13  1  0  0  0  0
M  END
> <DATABASE_ID>
MMDBc0000327

> <DATABASE_NAME>
MIME

> <SMILES>
N[C@@H](CCCNC(=N)N[C@@H](CC(O)=O)C(O)=O)C(O)=O

> <INCHI_IDENTIFIER>
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

> <INCHI_KEY>
KDZOASGQNOPSCU-WDSKDSINSA-N

> <FORMULA>
C10H18N4O6

> <MOLECULAR_WEIGHT>
290.2731

> <EXACT_MASS>
290.122634328

> <JCHEM_ACCEPTOR_COUNT>
10

> <JCHEM_ATOM_COUNT>
38

> <JCHEM_AVERAGE_POLARIZABILITY>
27.598868203245992

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
7

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
(2S)-2-{N'-[(4S)-4-amino-4-carboxybutyl]carbamimidamido}butanedioic acid

> <ALOGPS_LOGP>
-3.25

> <JCHEM_LOGP>
-5.803854917241661

> <ALOGPS_LOGS>
-2.80

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-1

> <JCHEM_PKA>
3.2332188556928654

> <JCHEM_PKA_STRONGEST_ACIDIC>
2.1362975685976218

> <JCHEM_PKA_STRONGEST_BASIC>
12.386778734858158

> <JCHEM_POLAR_SURFACE_AREA>
185.82999999999998

> <JCHEM_REFRACTIVITY>
75.3116

> <JCHEM_ROTATABLE_BOND_COUNT>
9

> <JCHEM_RULE_OF_FIVE>
0

> <ALOGPS_SOLUBILITY>
4.56e-01 g/l

> <JCHEM_TRADITIONAL_IUPAC>
argininosuccinic acid

> <JCHEM_VEBER_RULE>
0

$$$$

HMDB0000052
     RDKit          3D
Argininosuccinic acid
 38 37  0  0  0  0  0  0  0  0999 V2000
   -1.7537    1.1031    1.3975 N   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2635    0.1775    0.6303 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.0404    0.4135    0.1178 N   0  0  0  0  0  0  0  0  0  0  0  0
    1.1901   -0.2059    0.7203 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.4371    0.2245   -0.0285 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.6871   -0.3917    0.5559 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.8487    0.1301   -0.2961 C   0  0  2  0  0  0  0  0  0  0  0  0
    4.8279    1.5801   -0.1710 N   0  0  0  0  0  0  0  0  0  0  0  0
    4.6665   -0.2449   -1.7043 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.5216    0.6172   -2.6111 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.6470   -1.5800   -2.0915 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9982   -0.9834    0.3383 N   0  0  0  0  0  0  0  0  0  0  0  0
   -3.3774   -0.9865   -0.1533 C   0  0  1  0  0  0  0  0  0  0  0  0
   -4.2994   -0.3156    0.7965 C   0  0  0  0  0  0  0  0  0  0  0  0
   -5.6851   -0.3741    0.3185 C   0  0  0  0  0  0  0  0  0  0  0  0
   -6.0243   -1.0415   -0.6769 O   0  0  0  0  0  0  0  0  0  0  0  0
   -6.6664    0.3593    1.0221 O   0  0  0  0  0  0  0  0  0  0  0  0
   -3.3269   -0.2232   -1.4294 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2197    0.2718   -1.8493 O   0  0  0  0  0  0  0  0  0  0  0  0
   -4.4053    0.0151   -2.2435 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2599    1.9719    1.6448 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.1488    1.0446   -0.7029 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.3115    0.0766    1.7748 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.0887   -1.3229    0.6093 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.5294    1.3397    0.0610 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.3588   -0.0287   -1.0867 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.8150   -0.0578    1.5964 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.6341   -1.4934    0.4785 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.7924   -0.2551    0.1438 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.6312    1.9888   -0.7046 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.8561    1.8742    0.8224 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.4503   -2.0237   -2.5060 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5714   -1.9222    0.4668 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.6908   -2.0048   -0.3675 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.2551   -0.7723    1.8256 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.0580    0.7674    0.9202 H   0  0  0  0  0  0  0  0  0  0  0  0
   -6.7943    1.3501    0.8576 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.8335    0.9221   -2.3876 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  2  3
  2  3  1  0
  3  4  1  0
  4  5  1  0
  5  6  1  0
  6  7  1  0
  7  8  1  0
  7  9  1  0
  9 10  2  0
  9 11  1  0
  2 12  1  0
 12 13  1  0
 13 14  1  0
 14 15  1  0
 15 16  2  0
 15 17  1  0
 13 18  1  0
 18 19  2  0
 18 20  1  0
  1 21  1  0
  3 22  1  0
  4 23  1  0
  4 24  1  0
  5 25  1  0
  5 26  1  0
  6 27  1  0
  6 28  1  0
  7 29  1  1
  8 30  1  0
  8 31  1  0
 11 32  1  0
 12 33  1  0
 13 34  1  6
 14 35  1  0
 14 36  1  0
 17 37  1  0
 20 38  1  0
M  END

HEADER    PROTEIN                                 08-JUL-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   08-JUL-20         0                                  
HETATM    1  C   UNK     0      -6.671   0.000   0.000  0.00  0.00           C+0
HETATM    2  C   UNK     0      -5.337   0.770   0.000  0.00  0.00           C+0
HETATM    3  C   UNK     0      -4.003   0.000   0.000  0.00  0.00           C+0
HETATM    4  N   UNK     0      -2.670   0.770   0.000  0.00  0.00           N+0
HETATM    5  C   UNK     0      -1.336   0.000   0.000  0.00  0.00           C+0
HETATM    6  N   UNK     0      -0.002   0.770   0.000  0.00  0.00           N+0
HETATM    7  C   UNK     0       1.331   0.000   0.000  0.00  0.00           C+0
HETATM    8  C   UNK     0      -4.003  -1.540   0.000  0.00  0.00           C+0
HETATM    9  N   UNK     0      -1.336  -1.540   0.000  0.00  0.00           N+0
HETATM   10  C   UNK     0       2.665   0.770   0.000  0.00  0.00           C+0
HETATM   11  C   UNK     0       3.999   0.000   0.000  0.00  0.00           C+0
HETATM   12  C   UNK     0       5.333   0.770   0.000  0.00  0.00           C+0
HETATM   13  C   UNK     0       6.666   0.000   0.000  0.00  0.00           C+0
HETATM   14  N   UNK     0       5.333   2.310   0.000  0.00  0.00           N+0
HETATM   15  O   UNK     0      -2.670  -2.310   0.000  0.00  0.00           O+0
HETATM   16  O   UNK     0      -5.337  -2.310   0.000  0.00  0.00           O+0
HETATM   17  O   UNK     0      -8.000   0.778   0.000  0.00  0.00           O+0
HETATM   18  O   UNK     0      -6.675  -1.538   0.000  0.00  0.00           O+0
HETATM   19  O   UNK     0       6.667  -1.540   0.000  0.00  0.00           O+0
HETATM   20  O   UNK     0       8.000   0.770   0.000  0.00  0.00           O+0
CONECT    1    2   17   18                                                  
CONECT    2    1    3                                                       
CONECT    3    2    4    8                                                  
CONECT    4    3    5                                                       
CONECT    5    4    6    9                                                  
CONECT    6    5    7                                                       
CONECT    7    6   10                                                       
CONECT    8    3   15   16                                                  
CONECT    9    5                                                            
CONECT   10    7   11                                                       
CONECT   11   10   12                                                       
CONECT   12   13   14   11                                                  
CONECT   13   12   19   20                                                  
CONECT   14   12                                                            
CONECT   15    8                                                            
CONECT   16    8                                                            
CONECT   17    1                                                            
CONECT   18    1                                                            
CONECT   19   13                                                            
CONECT   20   13                                                            
MASTER        0    0    0    0    0    0    0    0   20    0   38    0
END

COMPND    HMDB0000052
HETATM    1  N1  UNL     1      -1.754   1.103   1.398  1.00  0.00           N  
HETATM    2  C1  UNL     1      -1.263   0.177   0.630  1.00  0.00           C  
HETATM    3  N2  UNL     1       0.040   0.413   0.118  1.00  0.00           N  
HETATM    4  C2  UNL     1       1.190  -0.206   0.720  1.00  0.00           C  
HETATM    5  C3  UNL     1       2.437   0.225  -0.028  1.00  0.00           C  
HETATM    6  C4  UNL     1       3.687  -0.392   0.556  1.00  0.00           C  
HETATM    7  C5  UNL     1       4.849   0.130  -0.296  1.00  0.00           C  
HETATM    8  N3  UNL     1       4.828   1.580  -0.171  1.00  0.00           N  
HETATM    9  C6  UNL     1       4.667  -0.245  -1.704  1.00  0.00           C  
HETATM   10  O1  UNL     1       4.522   0.617  -2.611  1.00  0.00           O  
HETATM   11  O2  UNL     1       4.647  -1.580  -2.092  1.00  0.00           O  
HETATM   12  N4  UNL     1      -1.998  -0.983   0.338  1.00  0.00           N  
HETATM   13  C7  UNL     1      -3.377  -0.986  -0.153  1.00  0.00           C  
HETATM   14  C8  UNL     1      -4.299  -0.316   0.796  1.00  0.00           C  
HETATM   15  C9  UNL     1      -5.685  -0.374   0.318  1.00  0.00           C  
HETATM   16  O3  UNL     1      -6.024  -1.041  -0.677  1.00  0.00           O  
HETATM   17  O4  UNL     1      -6.666   0.359   1.022  1.00  0.00           O  
HETATM   18  C10 UNL     1      -3.327  -0.223  -1.429  1.00  0.00           C  
HETATM   19  O5  UNL     1      -2.220   0.272  -1.849  1.00  0.00           O  
HETATM   20  O6  UNL     1      -4.405   0.015  -2.244  1.00  0.00           O  
HETATM   21  H1  UNL     1      -1.260   1.972   1.645  1.00  0.00           H  
HETATM   22  H2  UNL     1       0.149   1.045  -0.703  1.00  0.00           H  
HETATM   23  H3  UNL     1       1.312   0.077   1.775  1.00  0.00           H  
HETATM   24  H4  UNL     1       1.089  -1.323   0.609  1.00  0.00           H  
HETATM   25  H5  UNL     1       2.529   1.340   0.061  1.00  0.00           H  
HETATM   26  H6  UNL     1       2.359  -0.029  -1.087  1.00  0.00           H  
HETATM   27  H7  UNL     1       3.815  -0.058   1.596  1.00  0.00           H  
HETATM   28  H8  UNL     1       3.634  -1.493   0.479  1.00  0.00           H  
HETATM   29  H9  UNL     1       5.792  -0.255   0.144  1.00  0.00           H  
HETATM   30  H10 UNL     1       5.631   1.989  -0.705  1.00  0.00           H  
HETATM   31  H11 UNL     1       4.856   1.874   0.822  1.00  0.00           H  
HETATM   32  H12 UNL     1       5.450  -2.024  -2.506  1.00  0.00           H  
HETATM   33  H13 UNL     1      -1.571  -1.922   0.467  1.00  0.00           H  
HETATM   34  H14 UNL     1      -3.691  -2.005  -0.367  1.00  0.00           H  
HETATM   35  H15 UNL     1      -4.255  -0.772   1.826  1.00  0.00           H  
HETATM   36  H16 UNL     1      -4.058   0.767   0.920  1.00  0.00           H  
HETATM   37  H17 UNL     1      -6.794   1.350   0.858  1.00  0.00           H  
HETATM   38  H18 UNL     1      -4.834   0.922  -2.388  1.00  0.00           H  
CONECT    1    2    2   21
CONECT    2    3   12
CONECT    3    4   22
CONECT    4    5   23   24
CONECT    5    6   25   26
CONECT    6    7   27   28
CONECT    7    8    9   29
CONECT    8   30   31
CONECT    9   10   10   11
CONECT   11   32
CONECT   12   13   33
CONECT   13   14   18   34
CONECT   14   15   35   36
CONECT   15   16   16   17
CONECT   17   37
CONECT   18   19   19   20
CONECT   20   38
END

HMDB0000052
     RDKit          3D
Argininosuccinic acid
 38 37  0  0  0  0  0  0  0  0999 V2000
   -1.7537    1.1031    1.3975 N   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2635    0.1775    0.6303 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.0404    0.4135    0.1178 N   0  0  0  0  0  0  0  0  0  0  0  0
    1.1901   -0.2059    0.7203 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.4371    0.2245   -0.0285 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.6871   -0.3917    0.5559 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.8487    0.1301   -0.2961 C   0  0  2  0  0  0  0  0  0  0  0  0
    4.8279    1.5801   -0.1710 N   0  0  0  0  0  0  0  0  0  0  0  0
    4.6665   -0.2449   -1.7043 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.5216    0.6172   -2.6111 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.6470   -1.5800   -2.0915 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9982   -0.9834    0.3383 N   0  0  0  0  0  0  0  0  0  0  0  0
   -3.3774   -0.9865   -0.1533 C   0  0  1  0  0  0  0  0  0  0  0  0
   -4.2994   -0.3156    0.7965 C   0  0  0  0  0  0  0  0  0  0  0  0
   -5.6851   -0.3741    0.3185 C   0  0  0  0  0  0  0  0  0  0  0  0
   -6.0243   -1.0415   -0.6769 O   0  0  0  0  0  0  0  0  0  0  0  0
   -6.6664    0.3593    1.0221 O   0  0  0  0  0  0  0  0  0  0  0  0
   -3.3269   -0.2232   -1.4294 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2197    0.2718   -1.8493 O   0  0  0  0  0  0  0  0  0  0  0  0
   -4.4053    0.0151   -2.2435 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2599    1.9719    1.6448 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.1488    1.0446   -0.7029 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.3115    0.0766    1.7748 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.0887   -1.3229    0.6093 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.5294    1.3397    0.0610 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.3588   -0.0287   -1.0867 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.8150   -0.0578    1.5964 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.6341   -1.4934    0.4785 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.7924   -0.2551    0.1438 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.6312    1.9888   -0.7046 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.8561    1.8742    0.8224 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.4503   -2.0237   -2.5060 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5714   -1.9222    0.4668 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.6908   -2.0048   -0.3675 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.2551   -0.7723    1.8256 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.0580    0.7674    0.9202 H   0  0  0  0  0  0  0  0  0  0  0  0
   -6.7943    1.3501    0.8576 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.8335    0.9221   -2.3876 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  2  3
  2  3  1  0
  3  4  1  0
  4  5  1  0
  5  6  1  0
  6  7  1  0
  7  8  1  0
  7  9  1  0
  9 10  2  0
  9 11  1  0
  2 12  1  0
 12 13  1  0
 13 14  1  0
 14 15  1  0
 15 16  2  0
 15 17  1  0
 13 18  1  0
 18 19  2  0
 18 20  1  0
  1 21  1  0
  3 22  1  0
  4 23  1  0
  4 24  1  0
  5 25  1  0
  5 26  1  0
  6 27  1  0
  6 28  1  0
  7 29  1  1
  8 30  1  0
  8 31  1  0
 11 32  1  0
 12 33  1  0
 13 34  1  6
 14 35  1  0
 14 36  1  0
 17 37  1  0
 20 38  1  0
M  END

Synonyms

Value	Source
2-(Nomega-L-arginino)succinate	Kegg
L-Argininosuccinate	Kegg
L-Argininosuccinic acid	Kegg
L-Arginosuccinic acid	Kegg
2-(Nomega-L-arginino)succinic acid	Generator
L-Arginosuccinate	Generator
Argininosuccinate	Generator
2-(N(Omega)-L-arginine)succinate	HMDB
2-(N(Omega)-L-arginine)succinic acid	HMDB
2-(N(Omega)-L-arginino)succinate	HMDB
2-(N(Omega)-L-arginino)succinic acid	HMDB
2-(Nw-l-arginino)butanedioate	HMDB
2-(Nw-l-arginino)butanedioic acid	HMDB
Arginosuccinate	HMDB
Arginosuccinic acid	HMDB
ASA	HMDB
N(Omega)-(L-arginino)succinate	HMDB
N(Omega)-(L-arginino)succinic acid	HMDB
N-(((4-Amino-4-carboxybutyl)amino)iminomethyl)-L-aspartate	HMDB
N-(((4-Amino-4-carboxybutyl)amino)iminomethyl)-L-aspartic acid	HMDB
N-(L-Arginino) succinate	HMDB
N-(L-Arginino) succinic acid	HMDB
N-(L-Arginino)succinate	HMDB
N-(L-Arginino)succinic acid	HMDB
N-[(4-Amino-4-carboxybutyl)amidino]-L-aspartate	HMDB
N-[(4-Amino-4-carboxybutyl)amidino]-L-aspartic acid	HMDB
N-[[(4-Amino-4-carboxybutyl)amino]iminomethyl]-L-aspartate	HMDB
N-[[(4-Amino-4-carboxybutyl)amino]iminomethyl]-L-aspartic acid	HMDB
Acid, argininosuccinic	HMDB
N-(4-Amino-4-carboxybutyl)amidino-L-aspartic acid	HMDB

Molecular Formula

C₁₀H₁₈N₄O₆

Average Mass

290.2731

Monoisotopic Mass

290.122634328

IUPAC Name

(2S)-2-{N'-[(4S)-4-amino-4-carboxybutyl]carbamimidamido}butanedioic acid

Traditional Name

argininosuccinic acid

CAS Registry Number

Not Available

SMILES

N[C@@H](CCCNC(=N)N[C@@H](CC(O)=O)C(O)=O)C(O)=O

InChI Identifier

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

InChI Key

KDZOASGQNOPSCU-WDSKDSINSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as aspartic acid and derivatives. Aspartic acid and derivatives are compounds containing an aspartic acid or a derivative thereof resulting from reaction of aspartic acid at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Amino acids, peptides, and analogues

Direct Parent

Aspartic acid and derivatives

Alternative Parents

Substituents

Aspartic acid or derivatives
Alpha-amino acid
L-alpha-amino acid
Tricarboxylic acid or derivatives
Guanidine
Amino acid
Organic 1,3-dipolar compound
Propargyl-type 1,3-dipolar organic compound
Carboximidamide
Carboxylic acid
Hydrocarbon derivative
Organopnictogen compound
Organic oxygen compound
Primary amine
Organooxygen compound
Organonitrogen compound
Primary aliphatic amine
Carbonyl group
Amine
Organic nitrogen compound
Organic oxide
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

Other amino acids (C03406 )

Functional Ontology

Not Available

Physical Properties

State

Expected Solid

Predicted Properties

Property	Value	Source
Water Solubility	0.46 g/L	ALOGPS
logP	-3.2	ALOGPS
logP	-5.8	ChemAxon
logS	-2.8	ALOGPS
pKa (Strongest Acidic)	2.14	ChemAxon
pKa (Strongest Basic)	12.39	ChemAxon
Physiological Charge	-1	ChemAxon
Hydrogen Acceptor Count	10	ChemAxon
Hydrogen Donor Count	7	ChemAxon
Polar Surface Area	185.83 Å²	ChemAxon
Rotatable Bond Count	9	ChemAxon
Refractivity	75.31 m³·mol⁻¹	ChemAxon
Polarizability	27.6 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	No	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Spectra

Spectrum Type	Description	Splash Key	Deposition Date	View
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)	splash10-0007-0921100000-638b5887a29712423afd	2014-06-16	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-0007-8790000000-bc8aa39ecc71a1589298	2017-09-01	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (3 TMS) - 70eV, Positive	splash10-0006-4101900000-c606d87ec7ffb8ef2cfa	2017-10-06	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_4) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_5) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_6) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_7) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_2) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_3) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_4) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_5) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_6) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_7) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_8) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_9) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_10) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_11) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_12) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_2_13) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative	splash10-000i-0090000000-27e7b2225bca7ed0bfef	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative	splash10-00di-0390000000-b66871354ad66743ff5f	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative	splash10-001i-1910000000-777b6f2263b573f38bca	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative	splash10-001i-3900000000-26d6466903550c40fde6	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negative	splash10-001r-8900000000-4e5455a5af85574ac9df	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Positive	splash10-0006-0090000000-79b7ca9043632dc877e1	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Positive	splash10-0006-0590000000-8fb4f4625f241d387e92	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Positive	splash10-06di-3910000000-55530a4477a3fde8c1c1	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Positive	splash10-00di-9200000000-413c3029964c0386047a	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Positive	splash10-00di-9000000000-6ffc3b2b5da5f69de80b	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive	splash10-060r-0950000000-129ee81e1370707d975d	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive	splash10-0a4i-0910000000-62431d9967ced6c96b28	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive	splash10-03di-1980000000-27b5f442d411115ebaab	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive	splash10-0a4i-1900000000-b57bcbf0c07e921767af	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive	splash10-0006-3900000000-1fce5114c61349fc9065	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive	splash10-014i-0900000000-25c094a4a18cfe463b6b	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive	splash10-00di-9200000000-e518e242f12e95bbddf9	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-IT (LC/MSD Trap XCT, Agilent Technologies) , Positive	splash10-00di-9000000000-932dfd009a8e5c41ec38	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-000i-0090000000-27e7b2225bca7ed0bfef	2017-09-14	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-00dj-0190000000-8ba86e35661de03d1049	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-0092-2890000000-966d24fb4f820888318e	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-004i-4900000000-5200a801423dd1a4b7a6	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-00ds-0290000000-a3397e8c303fc7e6bd83	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-00e9-0940000000-a964172e915808d27899	2017-09-01	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-0ac3-8900000000-d4ac87f770202453bb7a	2017-09-01	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 600 MHz, H₂O, experimental)		2012-12-04	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 100 MHz, H₂O, predicted)		2022-08-17	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 100 MHz, H₂O, predicted)		2022-08-17	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, H₂O, predicted)		2022-08-17	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 1000 MHz, H₂O, predicted)		2022-08-17	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 200 MHz, H₂O, predicted)		2022-08-17	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 200 MHz, H₂O, predicted)		2022-08-17	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 300 MHz, H₂O, predicted)		2022-08-17	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 300 MHz, H₂O, predicted)		2022-08-17	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 400 MHz, H₂O, predicted)		2022-08-17	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 400 MHz, H₂O, predicted)		2022-08-17	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 500 MHz, H₂O, predicted)		2022-08-17	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 500 MHz, H₂O, predicted)		2022-08-17	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 600 MHz, H₂O, predicted)		2022-08-17	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 600 MHz, H₂O, predicted)		2022-08-17	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 700 MHz, H₂O, predicted)		2022-08-17	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 700 MHz, H₂O, predicted)		2022-08-17	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 800 MHz, H₂O, predicted)		2022-08-17	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 800 MHz, H₂O, predicted)		2022-08-17	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 900 MHz, H₂O, predicted)		2022-08-17	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 900 MHz, H₂O, predicted)		2022-08-17	View Spectrum
2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 600 MHz, H₂O, experimental)		2012-12-04	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm

Biospecimen Locations

Blood
Feces
Kidney
Placenta
Prostate
Urine

Tissue Locations

Kidney
Placenta
Prostate

Associated OMIM IDs

114500 (Colorectal cancer)
610247 (Eosinophilic esophagitis)

Human Proteins and Enzymes

Protein ID	Protein Name	Protein Type	Uniprot ID	Species
MMDBp0192229	Adenylosuccinate lyase	Enzyme	P30566	Homo sapiens
MMDBp0192297	Argininosuccinate synthase	Enzyme	P00966	Homo sapiens
MMDBp0192336	Argininosuccinate lyase	Enzyme	P04424	Homo sapiens
MMDBp0193702	Cholinephosphotransferase 1	Unknown	Q8WUD6	Homo sapiens
MMDBp0195860	Argininosuccinate synthase	Enzyme	Q5T6L4	Homo sapiens

Human Pathways

Pathways

Name	SMPDB/PathBank

Metabolic Reactions

Reactions

Reaction ID	Precursor	Product	Enzyme	Data Source	Reference
MMDBr0000332	L-Aspartic acid	Argininosuccinic acid	Not Available	VMH; KEGG	30371894
MMDBr0007051	L-Aspartic acid	Argininosuccinic acid	Not Available	VMH	30371894
MMDBr0007722	Argininosuccinic acid	L-Arginine	Not Available	VMH; KEGG	30371894

Health Effects and Bioactivity

Health Outcome/Bioactivity	Metabolite Response/Effect	Evidence Type	Host and Biospecimen	Data Source	Reference
Argininosuccinic aciduria	Increased	Association	Human Urine	HMDB	12408190
Argininosuccinic aciduria	Increased	Association	Human Blood	HMDB	12408190
Argininosuccinic aciduria	Increased	Association	Human Urine	HMDB	5075233
Argininemia		Association	Human Urine	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)

Microbial Sources

Superkingdom/Kingdom	Phylum	Total species	Hosts and Body Sites	Microbial Links
Bacteria	Actinobacteria	169	Human feces; Human pleural fluid plaque; Human dental plaque; Human sputum; Human ; Human nasopharyngeal swab; Human supragingival plaque; Human mucosa; Human subgingival plaque; Cattle	Indirect association between microbiota composition and metabolites measured in host biospecimen; Metabolic reconstruction
Bacteria	Firmicutes	414	Human ; Human mucosa; Human feces; Human saliva; Human sputum; Human stool; Pig ; Human subgingival plaque; Human urine; Cattle ; Catfish	Indirect association between microbiota composition and metabolites measured in host biospecimen; Metabolic reconstruction
Bacteria	Proteobacteria	518	Human feces; Human sputum; Human ; Human unknown; Pig ; Human pleural fluid plaque; Human dental plaque; Human nasal swab; Human stool; Human feces; pleural fluid plaque; bal; Cattle ; River trout ; Rat ; Sheep ; Dog ; Chicken ; Turkey	Metabolic reconstruction
Bacteria	Verrucomicrobia	1	Human ; Human feces	Metabolic reconstruction
Bacteria	Bacteroidetes	92	Human feces; Human appendix biopsy; Human ; Human stool; Human saliva; Human gastric fluid; Human urine	Metabolic reconstruction
Bacteria	Synergistetes	4	Human feces	Metabolic reconstruction
Archaea	Thaumarchaeota	1		Metabolic reconstruction
Bacteria	Fusobacteria	15	Human feces; Human	Metabolic reconstruction
Archaea	Euryarchaeota	7		Metabolic reconstruction
Bacteria	Tenericutes	2	Human feces	Metabolic reconstruction
Bacteria	Planctomycetes	2	Human feces	Metabolic reconstruction
Archaea	Crenarchaeota	1		Metabolic reconstruction
Bacteria	Cyanobacteria	35	Human
Bacteria	Acidobacteria	3
Bacteria	Aquificae	3
Archaea/Archaea	Euryarchaeota	29		Metabolic reconstruction
Bacteria	Spirochaetes	7	Pig ; Human
Bacteria	Chlorobi	10
Bacteria	Chloroflexi	9
Bacteria	Deinococcus-thermus	5
Bacteria	Dictyoglomi	2
Bacteria	Elusimicrobia	1
Bacteria	Gemmatimonadetes	1
Archaea/Archaea	Crenarchaeota	10		Metabolic reconstruction
Bacteria	Thermotogae	6
Eukaryota/Fungi	Ascomycota	5	Human feces; Human saliva; Human supragingival plaque	Unknown
Bacteria	nah	1
Archaea/Archaea	Thaumarchaeota	1		Metabolic reconstruction

Exposure Sources

Source Type	Source Sub-type	Species	Data Source	Reference

Host Biospecimen and Location

Host and Biospecimen	Status	Mean	Min	Max	Units	Age	Sex	Health Condition	Data Source	Reference
Human Feces	Detected but not Quantified								HMDB
Human Urine	Detected and Quantified	667			umol/mmol creatinine	Infant (0-1 year old)	Male	Argininosuccinic aciduria (ASL)	HMDB	12408190
Human Blood	Detected and Quantified	1515			uM	Infant (0-1 year old)	Male	Argininosuccinic aciduria (ASL)	HMDB	12408190
Human Urine	Detected and Quantified	1542			umol/mmol creatinine	Adult (>18 years old)	Both	Argininosuccinic aciduria (ASL)	HMDB	5075233
Human Blood	Detected and Quantified	300.0	270.0	458.0	uM	Children (1-13 years old)	Female	Argininosuccinyl-CoA lyase deficiency	HMDB	8582405
Human Urine	Detected and Quantified	0			umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	21312326
Human Blood	Detected and Quantified	<2			uM	Infant (0-1 year old)	Male	Normal	HMDB	12408190
Human Urine	Detected and Quantified	<4			umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	HMDB	12408190
Human Urine	Detected and Quantified		0	99	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	31506554
Human Urine	Detected and Quantified	0.65	0.0	1.3	umol/mmol creatinine	Children (1-13 years old)	Both	Argininemia	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Blood	Detected and Quantified		0	1	uM	Children (1 - 13 years old)	Both	Normal	HMDB	Argininosuccinate Lyase Deficiency. 2011 Feb 3 [Updated 2012 Feb 2]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. Available from: https://www.ncbi.nlm.nih.gov/books/NBK51784/GeneReviews®. Seattle (WA).
Human Blood	Detected and Quantified		0	5	uM	Newborn (0-30 days old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Blood	Detected and Quantified		0	5	uM	Infant (1 - 3 months old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Blood	Detected and Quantified		0	5	uM	Children (3 months - 6 years old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Blood	Detected and Quantified		0	5	uM	Children (6 - 18 years old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Urine	Detected and Quantified	<0.433			umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Human Urine	Detected and Quantified	<1.70			umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified	<2.71			umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified	<4.19			umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified	<5.43			umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified	<7.81			umol/mmol creatinine	Adolescent (13-18 years old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Urine	Detected and Quantified	<8.71			umol/mmol creatinine	Infant (0-1 year old)	Not Specified	Normal	HMDB	Mayo Clinic (https://neurology.testcatalog.org/show/AAPD)
Human Kidney	Detected but not Quantified					Not Specified	Not Specified	Normal	HMDB	34986597
Human Placenta	Detected but not Quantified					Not Specified	Not Specified	Normal	HMDB	34986597
Human Prostate	Detected but not Quantified					Not Specified	Not Specified	Normal	HMDB	34986597
Human Feces	Detected but not Quantified					Adult (>18 years old)	Both	Normal	HMDB	29808030
Human Urine	Detected but not Quantified					Not Specified	Male	Normal	HMDB	32822159
Human Urine	Detected but not Quantified					Adult (>18 years old)	Both	Normal	HMDB	32966057
Human Blood	Detected but not Quantified					Adult (>18 years old)	Both	Normal	HMDB	32966057

External Links

HMDB ID

HMDB0000052

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

KNApSAcK ID

Chemspider ID

KEGG Compound ID

BioCyc ID

BiGG ID

Wikipedia Link

Argininosuccinic acid

METLIN ID

5115

PubChem Compound

16950

PDB ID

Not Available

ChEBI ID

15682

Food Biomarker Ontology

Not Available

References

Synthesis Reference

Not Available

General References

Glick NR, Snodgrass PJ, Schafer IA: Neonatal argininosuccinic aciduria with normal brain and kidney but absent liver argininosuccinate lyase activity. Am J Hum Genet. 1976 Jan;28(1):22-30. [PubMed:174426 ]
Fleisher LD, Rassin DK, Desnick RJ, Salwen HR, Rogers P, Bean M, Gaull GE: Argininosuccinic aciduria: prenatal studies in a family at risk. Am J Hum Genet. 1979 Jul;31(4):439-45. [PubMed:484552 ]
Michels VV, Beaudet AL: Arginase deficiency in multiple tissues in argininemia. Clin Genet. 1978 Jan;13(1):61-7. doi: 10.1111/j.1399-0004.1978.tb04128.x. [PubMed:624188 ]
Pelli N, Fensom AH, Slade C, Boa F, Mieli-Vergani G, Vergani D: Argininosuccinate lyase: a new autoantigen in liver disease. Clin Exp Immunol. 1998 Dec;114(3):455-61. doi: 10.1046/j.1365-2249.1998.00754.x. [PubMed:9844057 ]
Wasant P, Srisomsap C, Liammongkolkul S, Svasti J: Urea cycle disorders in Thai infants: a report of 5 cases. J Med Assoc Thai. 2002 Aug;85 Suppl 2:S720-31. [PubMed:12403252 ]
Kleijer WJ, Garritsen VH, Linnebank M, Mooyer P, Huijmans JG, Mustonen A, Simola KO, Arslan-Kirchner M, Battini R, Briones P, Cardo E, Mandel H, Tschiedel E, Wanders RJ, Koch HG: Clinical, enzymatic, and molecular genetic characterization of a biochemical variant type of argininosuccinic aciduria: prenatal and postnatal diagnosis in five unrelated families. J Inherit Metab Dis. 2002 Sep;25(5):399-410. doi: 10.1023/a:1020108002877. [PubMed:12408190 ]
Reid Sutton V, Pan Y, Davis EC, Craigen WJ: A mouse model of argininosuccinic aciduria: biochemical characterization. Mol Genet Metab. 2003 Jan;78(1):11-6. doi: 10.1016/s1096-7192(02)00206-8. [PubMed:12559843 ]
Au WL, Lim TC, Seow DC, Koh PL, Loh NK, Lim MS, Tan IK, Yee WC: Serial diffusion-weighted magnetic resonance imaging in adult-onset citrullinaemia. J Neurol Sci. 2003 May 15;209(1-2):101-4. doi: 10.1016/s0022-510x(02)00439-2. [PubMed:12686410 ]
Cohen BD: Methyl group deficiency and guanidino production in uremia. Mol Cell Biochem. 2003 Feb;244(1-2):31-6. [PubMed:12701806 ]
Scaglia F, Brunetti-Pierri N, Kleppe S, Marini J, Carter S, Garlick P, Jahoor F, O'Brien W, Lee B: Clinical consequences of urea cycle enzyme deficiencies and potential links to arginine and nitric oxide metabolism. J Nutr. 2004 Oct;134(10 Suppl):2775S-2782S; discussion 2796S-2797S. doi: 10.1093/jn/134.10.2775S. [PubMed:15465784 ]
Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]

Showing metabocard for Argininosuccinic acid (MMDBc0000327)

MOL for #<Metabolite:0x00005634c2ab5070>

3D MOL for #<Metabolite:0x00005634c2ab5070>

3D SDF for #<Metabolite:0x00005634c2ab5070>

3D-SDF for #<Metabolite:0x00005634c2ab5070>

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Structure for #<Metabolite:0x00005634c2ab5070>

3D Structure for #<Metabolite:0x00005634c2ab5070>