CHEBI:16015
ChEBI
6022
PubChem-compound
O75746
UniProt
Q9UI32
UniProt
6262
PubChem-compound
Citrulline
937
ChemSpider
1.0
Ornithine carbamoyltransferase, mitochondrial
PW000183
PathWhiz
CHEBI:16134
ChEBI
2.0
L-Aspartic acid
SubPathwayOutput
439153
PubChem-compound
110-17-8
CAS
Arginase-1
50
ChemSpider
C00327
KEGG Compound
CHEBI:16027
ChEBI
1.0
OXALACETIC_ACID
BioCyc
ATP
BioCyc
PW000052
PathWhiz
Aspartate aminotransferase, mitochondrial
559142
ChemSpider
PW000046
PathWhiz
PW000043
PathWhiz
5746
ChemSpider
5745
ChemSpider
5742
ChemSpider
Ornithine carbamoyltransferase, mitochondrial
1.0
L-ASPARTATE
BioCyc
1143
ChemSpider
PW000160
PathWhiz
UREA
BioCyc
Transport139
false
L-Glutamine (→) Transport: Homo sapiens, Extracellular Space to Homo sapiens, Cell, Cytoplasm
LEFT_TO_RIGHT
1.0
L-Glutamic acid
ARG
BioCyc
5858
ChemSpider
Adenosine monophosphate
Carbon dioxide
Transport141
false
L-Alanine (→) Transport: Homo sapiens, Extracellular Space to Homo sapiens, Cell, Cytoplasm
LEFT_TO_RIGHT
5735
ChemSpider
1.0
L-CITRULLINE
BioCyc
PW000158
PathWhiz
945
ChemSpider
PW000152
PathWhiz
16950
PubChem-compound
PW000030
PathWhiz
1.0
L-Aspartic acid
PPI
BioCyc
HMDB0001096
HMDB
Arginase-1
1.0
C5H10N2O3
L-Glutamine
146.06914
CH4NO5P
Carbamoyl phosphate
140.98271
Neutral amino acid transporter A
L-Alanine
SubPathwayInput
Alanine aminotransferase 1
7664-38-2
CAS
1.0
6.0
1.0
C00009
KEGG Compound
C00008
KEGG Compound
L-ALPHA-ALANINE
BioCyc
C00001
KEGG Compound
1.0
C00122
KEGG Compound
56-41-7
CAS
CHEBI:18361
ChEBI
NADH
C00004
KEGG Compound
CHEBI:18367
ChEBI
C00003
KEGG Compound
1032
ChemSpider
1031
ChemSpider
C00002
KEGG Compound
1.0
Fumaric acid
SubPathwayInput
Carbamoyl-phosphate synthase [ammonia], mitochondrial
3.5.3.1
false
3.5.3.1
L-Arginine + Water → Ornithine + Urea
LEFT_TO_RIGHT
C6H13N3O3
Citrulline
175.09569
372-75-8
CAS
C21H27N7O14P2
NAD
663.10913
1.0
C03406
KEGG Compound
1.0
44367445
PubChem-compound
Adenosine triphosphate
C00011
KEGG Compound
1.0
HMDB0000902
HMDB
2.0
HMDB0000904
HMDB
C00014
KEGG Compound
Extracellular Space
C00013
KEGG Compound
Calcium-binding mitochondrial carrier protein Aralar1
Neutral amino acid transporter B(0)
Phosphate
C00022
KEGG Compound
56-65-5
CAS
53-84-9
CAS
C00020
KEGG Compound
C00026
KEGG Compound
C00025
KEGG Compound
CHEBI:26078
ChEBI
L-Glutamic acid
SubPathwayOutput
SubPathwayInput
SubPathwayInteraction675
SubPathway675Reaction
SubPathwayReaction
SubPathwayInteraction676
SubPathway676Reaction
SubPathwayReaction
SubPathwayInteraction674
SubPathwayReaction
SubPathway674Reaction
SubPathwayInteraction679
SubPathway679Reaction
SubPathwayReaction
SubPathwayInteraction677
SubPathwayReaction
SubPathway677Reaction
1.4.1.3
false
1.4.1.3
L-Glutamic acid + NAD + Water → Ammonia + NADH + Oxoglutaric acid
LEFT_TO_RIGHT
SubPathwayInteraction678
SubPathwayReaction
SubPathway678Reaction
9367
ChemSpider
C00036
KEGG Compound
328-50-7
CAS
SMILES
NC(=O)C1=CN(C=CC1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1O
HMDB0000134
HMDB
Argininosuccinate lyase
AMP
BioCyc
1.0
C00041
KEGG Compound
HMDB0000250
HMDB
SMILES
OP(O)(=O)OP(O)(O)=O
HMDB0001341
HMDB
Argininosuccinate synthase
1.0
L-Glutamine
C00049
KEGG Compound
3.5.1.2
false
3.5.1.2
L-Glutamine + Water → Ammonia + L-Glutamic acid
LEFT_TO_RIGHT
C00169
KEGG Compound
56-85-9
CAS
SMILES
NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O
Alanine aminotransferase 1
HMDB0000148
HMDB
Argininosuccinate lyase
CH4N2O
Urea
60.032364
1.0
SubPathwayInteraction698
SubPathway698Reaction
SubPathwayReaction
10197150
ChemSpider
4.3.2.1
false
4.3.2.1
2 Argininosuccinic acid → Fumaric acid + L-Arginine
LEFT_TO_RIGHT
SubPathwayInteraction699
SubPathway699Reaction
SubPathwayReaction
Ammonia
SubPathwayOutput
SubPathwayInput
FUM
BioCyc
SMILES
OC(=O)CC(=O)C(O)=O
7732-18-5
CAS
6.3.4.5
false
6.3.4.5
Adenosine triphosphate + Citrulline + L-Aspartic acid → Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate
LEFT_TO_RIGHT
Aspartate aminotransferase, mitochondrial
CHEBI:18012
ChEBI
CHEBI:16199
ChEBI
HMDB0001487
HMDB
SMILES
N[C@@H](CCCNC(N)=N)C(O)=O
61-19-8
CAS
1.0
SMILES
NC1=C2N=CN([C@@H]3O[C@H](COP(O)(O)=O)[C@@H](O)[C@H]3O)C2=NC=N1
1.0
C00062
KEGG Compound
SMILES
N[C@@H](CCCNC(=N)N[C@@H](CC(O)=O)C(O)=O)C(O)=O
Pyruvic acid
AMMONIA
BioCyc
SMILES
N
56-86-0
CAS
590-55-6
CAS
Glutamate dehydrogenase 1, mitochondrial
L-Aspartic acid
C10H16N5O13P3
Adenosine triphosphate
506.99576
CHEBI:17053
ChEBI
C00064
KEGG Compound
P24298
UniProt
NAD
2387-71-5
CAS
Pyrophosphate
2.6.1.2
false
2.6.1.2
L-Alanine + Oxoglutaric acid ↔ L-Glutamic acid + Pyruvic acid
REVERSIBLE
SMILES
CC(=O)C(O)=O
HMDB0000045
HMDB
1.0
HMDB0000161
HMDB
962
PubChem-compound
C3H7NO2
L-Alanine
89.047676
5800
ChemSpider
4.0
C00077
KEGG Compound
CO2
Carbon dioxide
43.98983
979
ChemSpider
Argininosuccinate synthase
Carbamoyl-phosphate synthase [ammonia], mitochondrial
1.0
Water
H3O4P
Phosphoric acid
97.9769
Glutamate Metabolism
SubPathway
HMDB0000051
HMDB
SMILES
N[C@@H](CCC(O)=O)C(O)=O
HMDB0002111
HMDB
HMDB0000294
HMDB
HMDB0000052
HMDB
Adenosine diphosphate
L-Glutamine
14265-44-2
CAS
PYRUVATE
BioCyc
58-64-0
CAS
Glutaminase liver isoform, mitochondrial
Mitochondrial ornithine transporter 1
C00086
KEGG Compound
Ornithine carbamoyltransferase, mitochondrial
C4H7NO4
L-Aspartic acid
133.0375
644102
PubChem-compound
L-Glutamine
SubPathwayOutput
SubPathwayInput
6083
PubChem-compound
Argininosuccinate lyase
Argininosuccinic acid
CHEBI:18050
ChEBI
H2O
Water
18.010565
1.0
Phosphoric acid
HMDB0000191
HMDB
NADH
BioCyc
NAD
BioCyc
0.0
Glutamate dehydrogenase 1, mitochondrial
56-84-8
CAS
1.0
Glutamate dehydrogenase 1, mitochondrial
970
PubChem-compound
SMILES
OC(=O)\C=C\C(O)=O
P04424
UniProt
3.0
HMDB0002142
HMDB
30572
ChemSpider
SubPathwayInteraction712
SubPathway712Reaction
SubPathwayReaction
SubPathwayInteraction713
SubPathwayReaction
SubPathway713Reaction
C4H4O5
Oxalacetic acid
132.00587
ReactionCatalysis18
ACTIVATION
SubPathwayInteraction710
SubPathway710Reaction
SubPathwayReaction
SubPathwayInteraction711
SubPathwayReaction
SubPathway711Reaction
SubPathwayInteraction716
SubPathwayReaction
SubPathway716Reaction
SubPathwayInteraction717
SubPathwayReaction
SubPathway717Reaction
Adenosine triphosphate
SubPathwayInteraction714
SubPathway714Reaction
SubPathwayReaction
Argininosuccinate synthase
SubPathwayInteraction715
SubPathway715Reaction
SubPathwayReaction
ReactionCatalysis12
ACTIVATION
1.0
SubPathwayInteraction718
SubPathway718Reaction
SubPathwayReaction
21883788
PubChem-compound
SMILES
O
C10H18N4O6
Argininosuccinic acid
290.12262
H3N
Ammonia
17.026548
57-13-6
CAS
C6H14N4O2
L-Arginine
174.11168
C10H14N5O7P
Adenosine monophosphate
347.06308
D-Arginine and D-Ornithine Metabolism
SubPathway
4.0
P00480
UniProt
1.0
SubPathwayInteraction701
SubPathway701Reaction
SubPathwayReaction
6992098
PubChem-compound
SubPathwayInteraction702
SubPathwayReaction
SubPathway702Reaction
SubPathwayInteraction700
SubPathway700Reaction
SubPathwayReaction
HMDB0000538
HMDB
SubPathwayInteraction705
SubPathwayReaction
SubPathway705Reaction
SubPathwayInteraction706
SubPathway706Reaction
SubPathwayReaction
1004
PubChem-compound
SubPathwayInteraction703
SubPathway703Reaction
SubPathwayReaction
SubPathwayInteraction704
SubPathwayReaction
SubPathway704Reaction
5961
PubChem-compound
SubPathwayInteraction709
SubPathwayReaction
SubPathway709Reaction
1.0
TransportCatalysis7
ACTIVATION
SubPathwayInteraction708
SubPathwayReaction
SubPathway708Reaction
Histidine Metabolism
SubPathway
Ammonia
1.0
328-42-7
CAS
51
PubChem-compound
Water
CHEBI:15846
ChEBI
P00367
UniProt
ADP
BioCyc
C3H4O3
Pyruvic acid
88.016045
CHEBI:15729
ChEBI
ReactionCatalysis137
ACTIVATION
Pyrimidine Metabolism
SubPathway
ReactionCatalysis38
ACTIVATION
Neutral amino acid transporter B(0)
Alanine Metabolism
SubPathway
Purine Metabolism
SubPathway
274
ChemSpider
C5H6O5
Oxoglutaric acid
146.02153
272
ChemSpider
Ornithine
SubPathwayInput
CHEBI:16908
ChEBI
Citric Acid Cycle
SubPathway
70-26-8
CAS
H3O4P
Phosphate
97.9769
6026
ChemSpider
Aspartate aminotransferase, mitochondrial
ReactionCatalysis43
ACTIVATION
Aspartate Metabolism
SubPathway
Q9Y619
UniProt
SMILES
NC(N)=O
2.1.3.3
false
2.1.3.3
Carbamoyl phosphate + Ornithine → Citrulline + Phosphate
LEFT_TO_RIGHT
Mitochondrion
TransportCatalysis103
ACTIVATION
TransportCatalysis101
ACTIVATION
C5H12N2O2
Ornithine
132.08987
Reaction78
false
L-Aspartic acid + Oxoglutaric acid ↔ L-Glutamic acid + Oxalacetic acid
REVERSIBLE
Citrulline
CHEBI:16977
ChEBI
HMDB0000208
HMDB
217
ChemSpider
58-68-4
CAS
Water
SMILES
OP(O)(O)=O
HMDB0000214
HMDB
SMILES
OC(=O)CCC(=O)C(O)=O
CHEBI:30915
ChEBI
124-38-9
CAS
L-Alanine
SMP0000046
SMPDB
Argininemia
SMP0000044
SMPDB
ReactionCatalysis2
ACTIVATION
HMDB0001429
HMDB
Arginase-1
SMP0000050
SMPDB
HMDB0000223
HMDB
SMILES
NCCC[C@H](N)C(O)=O
GO:0005758
GENE ONTOLOGY
GO:0005759
GENE ONTOLOGY
GLN
BioCyc
Ammonia Recycling
SubPathway
GLT
BioCyc
7664-41-7
CAS
Malate-Aspartate Shuttle
SubPathway
Gluconeogenesis
SubPathway
C4H4O4
Fumaric acid
116.010956
Glucose-Alanine Cycle
SubPathway
SMP0000057
SMPDB
SMP0000055
SMPDB
SMILES
O=C=O
SMILES
C[C@H](N)C(O)=O
2.0
5950
PubChem-compound
222
PubChem-compound
1.0
SMILES
OP(O)(O)=O
H4O7P2
Pyrophosphate
177.94322
C21H29N7O14P2
NADH
665.12476
6082
ChemSpider
1.0
5957
PubChem-compound
SMP0000067
SMPDB
L-Glutamic acid
Alanine aminotransferase 1
2.0
Ornithine
SMP0000072
SMPDB
HMDB0000243
HMDB
GO:0005739
GENE ONTOLOGY
Arginine and Proline Metabolism
SubPathway
GO:0005615
GENE ONTOLOGY
C10H15N5O10P2
Adenosine diphosphate
427.02942
GO:0005737
GENE ONTOLOGY
SMILES
N[C@@H](CC(O)=O)C(O)=O
1.0
Selenoamino Acid Metabolism
SubPathway
16059
ChemSpider
Porphyrin Metabolism
SubPathway
0.0
P05089
UniProt
SMP0000009
SMPDB
2.0
SMP0000128
SMPDB
SMP0000129
SMPDB
SMP0000127
SMPDB
SMILES
N[C@@H](CCCNC(N)=O)C(O)=O
TransportCatalysis12
ACTIVATION
SMILES
NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](COP([O-])(=O)OP(O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C2N=CN=C3N)[C@@H](O)[C@H]1O
CPD-8587
BioCyc
5682
ChemSpider
CHEBI:15682
ChEBI
Oxalacetic acid
SubPathwayInput
P43007
UniProt
1.0
Mitochondrial ornithine transporter 1
Mitochondrial Matrix
Urea
14000-31-8
CAS
CARBAMOYL-P
BioCyc
Neutral amino acid transporter A
SMILES
NC(=O)OP(O)(O)=O
Cytoplasm
1.0
1.0
CHEBI:15422
ChEBI
SMP0000029
SMPDB
1.0
Homo sapiens
388299
ChemSpider
127-17-3
CAS
Calcium-binding mitochondrial carrier protein Aralar1
28782
PubChem-compound
SMP0000024
SMPDB
C5H9NO4
L-Glutamic acid
147.05316
SMP0000020
SMPDB
33032
PubChem-compound
L-Arginine
SubPathwayInput
Transport39
false
Ornithine (→), Citrulline (←) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Cell, Mitochondrial Matrix
LEFT_TO_RIGHT
CHEBI:16526
ChEBI
Transport31
false
L-Aspartic acid (→), L-Glutamic acid (←) Transport: Homo sapiens, Cell, Mitochondrial Matrix to Homo sapiens, Cell, Mitochondrial Intermembrane Space
LEFT_TO_RIGHT
CHEBI:16761
ChEBI
SMP0000036
SMPDB
9606
TAXONOMY
2-KETOGLUTARATE
BioCyc
Oxoglutaric acid
SubPathwayOutput
SubPathwayInput
5893
PubChem-compound
1176
PubChem-compound
L-Alanine
PW000019
PathWhiz
Mitochondrial Intermembrane Space
CHEBI:32816
ChEBI
1.0
CHEBI:15377
ChEBI
278
PubChem-compound
CHEBI:16467
ChEBI
PW000010
PathWhiz
CHEBI:16349
ChEBI
6.3.4.16
false
6.3.4.16
2 Adenosine triphosphate + Ammonia + Carbon dioxide + Water → 2 Adenosine diphosphate + Carbamoyl phosphate + Phosphoric acid
LEFT_TO_RIGHT
SMILES
NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O
P31327
UniProt
1.0
CHEBI:17672
ChEBI
280
PubChem-compound
CHEBI:30744
ChEBI
PW000009
PathWhiz
PW000007
PathWhiz
Carbamoyl phosphate
P00966
UniProt
PW000002
PathWhiz
ReactionCatalysis136
ACTIVATION
PW000001
PathWhiz
ReactionCatalysis133
ACTIVATION
PW000005
PathWhiz
ReactionCatalysis372
ACTIVATION
PW000003
PathWhiz
SMILES
N[C@@H](CCC(N)=O)C(O)=O
Q15758
UniProt
1060
PubChem-compound
1061
PubChem-compound
Glutaminase liver isoform, mitochondrial
74-79-3
CAS
SMP0000357
SMPDB
HMDB0001967
HMDB
1.0
HMDB0000517
HMDB
P00505
UniProt
HMDB0000641
HMDB
L-ORNITHINE
BioCyc
3.0