CHEBI:16015
ChEBI
2.0
Ornithine carbamoyltransferase, mitochondrial
1.0
439153
PubChem-compound
CHEBI:27391
ChEBI
50
ChemSpider
CHEBI:16027
ChEBI
OXALACETIC_ACID
BioCyc
Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
Guanidoacetic acid
559142
ChemSpider
Ornithine aminotransferase, mitochondrial
Q5T160
UniProt
P29475
UniProt
BTO:0000759
BRENDA TISSUE ONTOLOGY
5745
ChemSpider
5742
ChemSpider
Ornithine carbamoyltransferase, mitochondrial
L-ASPARTATE
BioCyc
1143
ChemSpider
CHEBI:16474
ChEBI
Aspartate aminotransferase, cytoplasmic
Carbon dioxide
UREA
BioCyc
TransportCatalysis5387
ACTIVATION
TransportCatalysis5388
ACTIVATION
Phosphocreatine
L-CITRULLINE
BioCyc
1.0
CHEBI:16480
ChEBI
CHEBI:16240
ChEBI
P04181
UniProt
L-4-Hydroxyglutamate semialdehyde
1-Pyrroline-5-carboxylic acid
HMDB0001096
HMDB
L-Arginyl-tRNA(Arg)
1.0
C5H9NO4
L-4-Hydroxyglutamate semialdehyde
147.05316
CH4NO5P
Carbamoyl phosphate
140.98271
979-92-0
CAS
SUC
BioCyc
Adenosine monophosphate
7664-38-2
CAS
2.0
HMDB0006556
HMDB
6.0
1.0
1.0
C00009
KEGG Compound
1159
ChemSpider
C00008
KEGG Compound
C00007
KEGG Compound
C00006
KEGG Compound
C05938
KEGG Compound
CHEBI:18009
ChEBI
C00001
KEGG Compound
Q7Z4N8
UniProt
C05933
KEGG Compound
C00005
KEGG Compound
C00004
KEGG Compound
C00003
KEGG Compound
C00002
KEGG Compound
2485-33-8
CAS
496-92-4
CAS
Pyrophosphate
Carbamoyl-phosphate synthase [ammonia], mitochondrial
N(omega)-Hydroxyarginine
3.5.3.1
false
3.5.3.1
L-Arginine + Water → Ornithine + Urea
LEFT_TO_RIGHT
C00019
KEGG Compound
352-97-6
CAS
1.0
ReactionCatalysis2194
ACTIVATION
ReactionCatalysis2193
ACTIVATION
C00011
KEGG Compound
2.0
1.0
C00014
KEGG Compound
C00013
KEGG Compound
10102-43-9
CAS
1-Pyrroline-5-carboxylic acid
ReactionCatalysis2196
ACTIVATION
ReactionCatalysis2195
ACTIVATION
1.0
ReactionCatalysis2198
ACTIVATION
ReactionCatalysis2197
ACTIVATION
ReactionCatalysis2199
ACTIVATION
H
Hydrogen Ion
1.007825
Phosphate
56-65-5
CAS
C00021
KEGG Compound
C00020
KEGG Compound
53-84-9
CAS
C00027
KEGG Compound
C00026
KEGG Compound
C00025
KEGG Compound
CHEBI:26078
ChEBI
1.0
1.0
L-Glutamic acid
Oxygen
1.4.1.3
false
1.4.1.3
L-Glutamic acid + NAD + Water → Ammonia + NADH + Oxoglutaric acid
LEFT_TO_RIGHT
CHEBI:29171
ChEBI
C5H7NO3
1-Pyrroline-4-hydroxy-2-carboxylate
129.04259
C01127
KEGG Compound
C00037
KEGG Compound
C00036
KEGG Compound
HMDB0000134
HMDB
SMILES
OC(=O)CCC(O)=O
1.0
L-Prolyl-tRNA(Pro)
L-Arginyl-tRNA(Arg)
tRNA(Pro)
tRNA(Arg)
CHEBI:29177
ChEBI
SMILES
OP(O)(=O)OP(O)(O)=O
4-Hydroxyproline
128566
ChemSpider
C00042
KEGG Compound
Water
C00049
KEGG Compound
1.2.1.88
false
1.2.1.88
1-Pyrroline-5-carboxylic acid + NAD + 2 Water ↔ L-Glutamic acid + NADH
REVERSIBLE
SMILES
NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O
Reaction2298
false
L-Proline → D-Proline
LEFT_TO_RIGHT
1.0
1.4.3.3
false
1.4.3.3
D-Proline + Oxygen + Water → 1-Pyrroline-2-carboxylic acid + Ammonia + Hydrogen peroxide
LEFT_TO_RIGHT
HMDB0003411
HMDB
HMDB0000148
HMDB
CH4N2O
Urea
60.032364
784
PubChem-compound
4.3.2.1
false
4.3.2.1
2 Argininosuccinic acid → Fumaric acid + L-Arginine
LEFT_TO_RIGHT
FUM
BioCyc
SMILES
OC(=O)CC(=O)C(O)=O
6.3.4.5
false
6.3.4.5
Adenosine triphosphate + Citrulline + L-Aspartic acid → Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate
LEFT_TO_RIGHT
CHEBI:18012
ChEBI
C03564
KEGG Compound
SMILES
N[C@@H](CCCNC(N)=N)C(O)=O
HMDB0001487
HMDB
Bifunctional glutamate/proline--tRNA ligase
1.0
C00062
KEGG Compound
SMILES
OC(=O)C1CCC=N1
CHEBI:29154
ChEBI
Adenosine diphosphate
C10H16N5O13P3
Adenosine triphosphate
506.99576
BiologicalState13
Homo sapiens, Liver
NAD
Pyrophosphate
C01157
KEGG Compound
HMDB0002104
HMDB
1.0
P50440
UniProt
HMDB0000162
HMDB
962
PubChem-compound
31983
ChemSpider
Probable arginine--tRNA ligase, mitochondrial
C00077
KEGG Compound
2.0
C01165
KEGG Compound
CO2
Carbon dioxide
43.98983
Proline dehydrogenase 1, mitochondrial
145742
PubChem-compound
C00080
KEGG Compound
2139-03-9
CAS
SMILES
O=O
HMDB0002111
HMDB
14265-44-2
CAS
1.0
9054-77-7
CAS
C00086
KEGG Compound
1.0
CPD-1128
BioCyc
644102
PubChem-compound
L-Arginine
1.0
C5H9NO5
4-Hydroxy-L-glutamic acid
163.04807
H2O2
Hydrogen peroxide
34.005478
HMDB0000191
HMDB
Ammonia
NADH
BioCyc
1.0
CREATINE
BioCyc
977
PubChem-compound
NADPH
Bifunctional glutamate/proline--tRNA ligase
1.0
970
PubChem-compound
C5H9NO3
4-Hydroxyproline
131.05824
SMILES
OC1CC(N=C1)C(O)=O
NAD
3.0
HMDB0002142
HMDB
P07814
UniProt
30572
ChemSpider
CPD-656
BioCyc
17215925
ChemSpider
SMILES
N[C@@H](C[C@@H](O)C(O)=O)C(O)=O
Argininosuccinate synthase
Carbamoyl-phosphate synthase [ammonia], mitochondrial
4-Hydroxy-L-glutamic acid
21883788
PubChem-compound
SMILES
OO
C10H18N4O6
Argininosuccinic acid
290.12262
H3N
Ammonia
17.026548
C10H14N5O7P
Adenosine monophosphate
347.06308
599
PubChem-compound
P00480
UniProt
1.0
HMDB0000538
HMDB
Ammonia
SMILES
O[C@H]1CN[C@@H](C1)C(O)=O
Water
51
PubChem-compound
C4H9N3O2
Creatine
131.06947
5460078
PubChem-compound
CHEBI:15729
ChEBI
167744
ChemSpider
SMILES
OC1CN=C(C1)C(O)=O
1.0
274
ChemSpider
272
ChemSpider
Ornithine
SubPathwayInteraction840
SubPathwayReaction
SubPathway840Reaction
Q14353
UniProt
SubPathwayInteraction842
SubPathway842Reaction
SubPathwayReaction
SubPathwayInteraction843
SubPathway843Reaction
SubPathwayReaction
H3O4P
Phosphate
97.9769
485-80-3
CAS
2.0
Adenosine triphosphate
1.0
Q9Y619
UniProt
SMILES
NC(N)=O
Q96C36
UniProt
SubPathwayInteraction7
SubPathway7Reaction
SubPathwayReaction
NADPH
BioCyc
Citrulline
S-Adenosylhomocysteine
CPD-6641
BioCyc
DELTA1-PYRROLINE_2-CARBOXYLATE
BioCyc
217
ChemSpider
Pyrroline-5-carboxylate reductase 2
58-68-4
CAS
2.0
C4H10N3O5P
Phosphocreatine
211.03581
SMILES
OP(O)(O)=O
C04281
KEGG Compound
C04282
KEGG Compound
CHEBI:30915
ChEBI
4-Hydroxyproline
l-delta(1)-pyrroline_5-carboxylate
BioCyc
C2H5NO2
Glycine
75.03203
Ornithine
SubPathwayOutput
GO:0005764
GENE ONTOLOGY
344-25-2
CAS
HMDB0001429
HMDB
Arginase-1
Liver
C3H7N3O2
Guanidoacetic acid
117.053825
GO:0005759
GENE ONTOLOGY
C4H4O4
Fumaric acid
116.010956
SMP0000057
SMPDB
SMP0000055
SMPDB
586
PubChem-compound
CHEBI:15741
ChEBI
587
PubChem-compound
SMILES
O=C=O
D-4-HYDROXY-2-KETO-GLUTARATE
BioCyc
1110
PubChem-compound
C21H29N7O14P2
NADH
665.12476
Glycine amidinotransferase, mitochondrial
1.0
5957
PubChem-compound
2.0
1.0
1.0
HMDB0000123
HMDB
GO:0005739
GENE ONTOLOGY
GO:0005737
GENE ONTOLOGY
2.0
HMDB0000128
HMDB
1.0
16059
ChemSpider
147-85-3
CAS
C5H6O6
4-Hydroxy-2-oxoglutaric acid
162.01643
Creatine
P05089
UniProt
2.0
1.0
NADH
1.0
1.0
5682
ChemSpider
2.7.3.2
false
2.7.3.2
Adenosine triphosphate + Creatine → Adenosine diphosphate + Phosphocreatine
LEFT_TO_RIGHT
Mitochondrial Matrix
14000-31-8
CAS
Urea
L-Glutamic γ-semialdehyde
Prolyl 4-hydroxylase subunit alpha-3
P30038
UniProt
67-07-2
CAS
5675
ChemSpider
1078
ChemSpider
389057
ChemSpider
NITRIC-OXIDE
BioCyc
HMDB0000939
HMDB
Carbon dioxide
CHEBI:1372
ChEBI
SMILES
NC(=O)OP(O)(O)=O
22573-88-2
CAS
Creatine kinase B-type
28782
PubChem-compound
1.2.1.88
false
1.2.1.88
L-Glutamic γ-semialdehyde + NAD + Water → Hydrogen Ion + L-Glutamic acid + NADH
LEFT_TO_RIGHT
1.0
SMP0000020
SMPDB
C5H9NO4
L-Glutamic acid
147.05316
Creatine kinase B-type
33032
PubChem-compound
L-Arginine
SubPathwayInput
4.0
7782-44-7
CAS
CHEBI:30923
ChEBI
Transport14571
false
Ornithine (→) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Cell, Mitochondrial Matrix
LEFT_TO_RIGHT
Transport14570
false
Citrulline (→) Transport: Homo sapiens, Cell, Mitochondrial Matrix to Homo sapiens, Cell, Cytoplasm
LEFT_TO_RIGHT
SMP0000036
SMPDB
9606
TAXONOMY
Nitric oxide
2-KETOGLUTARATE
BioCyc
HMDB0000725
HMDB
Guanidinoacetate N-methyltransferase
1.0
1176
PubChem-compound
110-15-6
CAS
Phosphoric acid
Nitric oxide synthase, brain
Oxoglutaric acid
1.14.13.39
false
1.14.13.39
2 L-Arginine + 3 NADPH + 4 Oxygen → 2 N(omega)-Hydroxyarginine + 3 NADP + 2 Nitric oxide + 4 Water
LEFT_TO_RIGHT
PW000019
PathWhiz
Reaction2305
false
4-Hydroxy-L-glutamic acid + Oxalacetic acid → 4-Hydroxy-2-oxoglutaric acid + L-Aspartic acid
LEFT_TO_RIGHT
1.5.5.2
false
1.5.5.2
4-Hydroxy-L-glutamic acid ↔ L-4-Hydroxyglutamate semialdehyde
REVERSIBLE
C6H14N4O3
N(omega)-Hydroxyarginine
190.1066
1.2.1.88
false
1.2.1.88
4-Hydroxy-L-glutamic acid + NAD + Water → L-4-Hydroxyglutamate semialdehyde + NADPH
LEFT_TO_RIGHT
1.4.3.3
false
1.4.3.3
4-Hydroxyproline + Oxygen + Water → 1-Pyrroline-4-hydroxy-2-carboxylate + Ammonia + Hydrogen peroxide
LEFT_TO_RIGHT
1.2.1.88
false
1.2.1.88
NAD + Pyrroline hydroxycarboxylic acid + Water → 4-Hydroxy-L-glutamic acid + NADPH
LEFT_TO_RIGHT
CHEBI:15377
ChEBI
Cell Membrane
1.0
CHEBI:15379
ChEBI
PW000010
PathWhiz
CHEBI:16467
ChEBI
CHEBI:15378
ChEBI
SMILES
[H+]
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
51-35-4
CAS
P31327
UniProt
389257
ChemSpider
NADPH
C00763
KEGG Compound
Ornithine aminotransferase, mitochondrial
CHEBI:30744
ChEBI
Carbamoyl phosphate
PW000001
PathWhiz
SMILES
N[C@@H](C[C@@H](O)C=O)C(O)=O
PW000005
PathWhiz
1196
PubChem-compound
C00533
KEGG Compound
1.0
2.0
1.0
1.0
74-79-3
CAS
CHEBI:16680
ChEBI
HMDB0000517
HMDB
C00300
KEGG Compound
2906-39-0
CAS
L-Proline
1.0
3.0
Prolyl 4-hydroxylase subunit alpha-3
5605
ChemSpider
1.0
6022
PubChem-compound
7722-84-1
CAS
1.0
Citrulline
6262
PubChem-compound
937
ChemSpider
127983
ChemSpider
CHEBI:16134
ChEBI
L-Aspartic acid
SubPathwayOutput
HMDB0003125
HMDB
110-17-8
CAS
HMDB0002273
HMDB
HMDB0006875
HMDB
Arginase-1
HMDB0001185
HMDB
C00327
KEGG Compound
1.0
tRNA(Arg)
389693
ChemSpider
Pyrroline hydroxycarboxylic acid
389691
ChemSpider
ATP
BioCyc
CHEBI:17232
ChEBI
Aspartate aminotransferase, cytoplasmic
D-Proline
HMDB0003378
HMDB
Hydrogen peroxide
Aspartate aminotransferase, cytoplasmic
PRO
BioCyc
HMDB0004224
HMDB
Glycine amidinotransferase, mitochondrial
CHEBI:17203
ChEBI
C15H23N6O5S
S-Adenosylmethionine
399.14505
1-Pyrroline-2-carboxylic acid
1.0
CHEBI:16352
ChEBI
1.0
Pyrroline hydroxycarboxylic acid
952
ChemSpider
440046
PubChem-compound
Proline dehydrogenase 1, mitochondrial
440282
PubChem-compound
5858
ChemSpider
ARG
BioCyc
Adenosine monophosphate
C00581
KEGG Compound
945
ChemSpider
16950
PubChem-compound
1010
ChemSpider
1.0
Pyrroline-5-carboxylate reductase 2
1.0
PPI
BioCyc
Arginase-1
HMDB0002070
HMDB
SMILES
C[S+](CC[C@H](N)C(O)=O)C[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC2=C1N=CN=C2N
Cytosol
C14H20N6O5S
S-Adenosylhomocysteine
384.12158
193305
PubChem-compound
1.0
2.1.4.1
false
2.1.4.1
Glycine + L-Arginine ↔ Guanidoacetic acid + Ornithine
REVERSIBLE
2.1.1.2
false
2.1.1.2
Guanidoacetic acid + S-Adenosylmethionine → Creatine + S-Adenosylhomocysteine
LEFT_TO_RIGHT
C02305
KEGG Compound
C00122
KEGG Compound
1.0
1030
ChemSpider
CHEBI:18361
ChEBI
NADH
CHEBI:18366
ChEBI
CHEBI:18367
ChEBI
1032
ChemSpider
1.0
Fumaric acid
SubPathwayInput
Carbamoyl phosphate
C6H13N3O3
Citrulline
175.09569
372-75-8
CAS
C03406
KEGG Compound
C21H27N7O14P2
NAD
663.10913
Bifunctional glutamate/proline--tRNA ligase
L-Arginine
44367445
PubChem-compound
Adenosine triphosphate
2.0
HMDB0000902
HMDB
HMDB0000904
HMDB
Prolyl 4-hydroxylase
L-Proline
NAD(P)
BioCyc
SMILES
N[C@@H](CCCNC(=N)NO)C(O)=O
388301
ChemSpider
Nitric-oxide synthase, brain
C00148
KEGG Compound
C5H9NO2
D-Proline
115.06333
9367
ChemSpider
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
Argininosuccinate lyase
HMDB0000254
HMDB
AMP
BioCyc
GUANIDOACETIC_ACID
BioCyc
Oxalacetic acid
HYDROGEN-PEROXIDE
BioCyc
Adenosine diphosphate
HMDB0000250
HMDB
HMDB0001341
HMDB
Argininosuccinate synthase
1.0
4-Hydroxy-L-glutamic acid
C00169
KEGG Compound
Ornithine aminotransferase, mitochondrial
763
ChemSpider
1.0
Argininosuccinate lyase
C5H7NO2
1-Pyrroline-2-carboxylic acid
113.047676
1.0
1.0
1.0
SMILES
OC(CC(=O)C(O)=O)C(O)=O
10197150
ChemSpider
Pyrroline hydroxycarboxylic acid
1.0
Ammonia
7732-18-5
CAS
CHEBI:16199
ChEBI
Glycine amidinotransferase, mitochondrial
CHEBI:17287
ChEBI
61-19-8
CAS
NAD
SMILES
NC1=C2N=CN([C@@H]3O[C@H](COP(O)(O)=O)[C@@H](O)[C@H]3O)C2=NC=N1
Creatine kinase B-type
D-amino-acid oxidase
SMILES
N[C@@H](CCCNC(=N)N[C@@H](CC(O)=O)C(O)=O)C(O)=O
HMDB0001369
HMDB
AMMONIA
BioCyc
SMILES
N
56-86-0
CAS
590-55-6
CAS
Glutamate dehydrogenase 1, mitochondrial
2.0
CHEBI:17053
ChEBI
56-40-6
CAS
NO
Nitric oxide
29.997988
2387-71-5
CAS
HMDB0001377
HMDB
HMDB0000045
HMDB
SMILES
CN(CC(O)=O)C(N)=N
53054-07-2
CAS
2.0
C5H9NO2
L-Proline
115.06333
5800
ChemSpider
4.0
979
ChemSpider
Argininosuccinate synthase
730
ChemSpider
HMDB0002234
HMDB
Water
H3O4P
Phosphoric acid
97.9769
4-Hydroxy-2-oxoglutaric acid
HMDB0000051
HMDB
SMILES
N[C@@H](CCC(O)=O)C(O)=O
HMDB0000052
HMDB
HMDB0000294
HMDB
58-64-0
CAS
1.0
1.0
Mitochondrial ornithine transporter 1
C04322
KEGG Compound
D-amino-acid oxidase
Pyrroline-5-carboxylate reductase 2
Nitric oxide synthase, brain
Ornithine carbamoyltransferase, mitochondrial
C4H7NO4
L-Aspartic acid
133.0375
1.0
HMDB0000064
HMDB
L-GLUTAMATE_GAMMA-SEMIALDEHYDE
BioCyc
57-00-1
CAS
1.0
6083
PubChem-compound
Argininosuccinate lyase
C5H9NO3
L-Glutamic γ-semialdehyde
131.05824
25246222
PubChem-compound
22833512
PubChem-compound
L-Aspartic acid
SMILES
CN(CC(O)=O)C(=N)NP(O)(O)=O
Argininosuccinic acid
H2O
Water
18.010565
Oxygen
SMILES
NCC(O)=O
P12277
UniProt
Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
NAD
BioCyc
HMDB0059597
HMDB
Guanidinoacetate N-methyltransferase
56-84-8
CAS
Glutamate dehydrogenase 1, mitochondrial
Glutamate dehydrogenase 1, mitochondrial
1.0
SMILES
OC(=O)\C=C\C(O)=O
743
ChemSpider
3.0
P04424
UniProt
SMILES
NC(=N)NCC(O)=O
D-amino-acid oxidase
ReactionCatalysis153
ACTIVATION
ReactionCatalysis18
ACTIVATION
ReactionCatalysis151
ACTIVATION
C4H4O5
Oxalacetic acid
132.00587
ReactionCatalysis152
ACTIVATION
SMILES
N[C@@H](CCC=O)C(O)=O
ReactionCatalysis150
ACTIVATION
Adenosine triphosphate
ReactionCatalysis12
ACTIVATION
1.0
C5H7NO2
1-Pyrroline-5-carboxylic acid
113.047676
SMILES
O
CHEBI:43088
ChEBI
57-13-6
CAS
C6H14N4O2
L-Arginine
174.11168
D-Arginine and D-Ornithine Metabolism
SubPathway
4.0
ReactionCatalysis146
ACTIVATION
ReactionCatalysis147
ACTIVATION
Reaction649
true
L-Glutamic γ-semialdehyde ↔ 1-Pyrroline-5-carboxylic acid
REVERSIBLE
ReactionCatalysis145
ACTIVATION
6992098
PubChem-compound
ReactionCatalysis143
ACTIVATION
ReactionCatalysis140
ACTIVATION
ReactionCatalysis141
ACTIVATION
1004
PubChem-compound
1.0
HMDB0001511
HMDB
328-42-7
CAS
CHEBI:15846
ChEBI
P00367
UniProt
ADP
BioCyc
ReactionCatalysis139
ACTIVATION
ReactionCatalysis137
ACTIVATION
ReactionCatalysis138
ACTIVATION
ReactionCatalysis38
ACTIVATION
4.0
1.0
1.0
Lysosome
53-59-8
CAS
Reaction658
true
Pyrroline hydroxycarboxylic acid ↔ L-4-Hydroxyglutamate semialdehyde
REVERSIBLE
2.6.1.13
false
2.6.1.13
Ornithine + Oxoglutaric acid ↔ L-Glutamic acid + L-Glutamic γ-semialdehyde
REVERSIBLE
1.5.5.2
false
1.5.5.2
4-Hydroxyproline ↔ Pyrroline hydroxycarboxylic acid
REVERSIBLE
440849
PubChem-compound
SMILES
OC(=O)[C@H]1CCCN1
1038
PubChem-compound
CHEBI:27809
ChEBI
CHEBI:36761
ChEBI
4.0
GO:0005829
GENE ONTOLOGY
Alanine Metabolism
SubPathway
CHEBI:18095
ChEBI
C5H6O5
Oxoglutaric acid
146.02153
1.5.1.2
false
1.5.1.2
4-Hydroxyproline + NAD → NADH + Pyrroline hydroxycarboxylic acid
LEFT_TO_RIGHT
6.1.1.17
false
6.1.1.17
Adenosine triphosphate + L-Proline + tRNA(Pro) → Adenosine monophosphate + L-Prolyl-tRNA(Pro) + Pyrophosphate
LEFT_TO_RIGHT
1.5.5.2
false
1.5.5.2
1-Pyrroline-5-carboxylic acid ↔ L-Proline
REVERSIBLE
1.5.1.2
false
1.5.1.2
L-Proline + NAD → 1-Pyrroline-5-carboxylic acid + NADH
LEFT_TO_RIGHT
CHEBI:16908
ChEBI
L-ERYTHRO-4-HYDROXY-GLUTAMATE
BioCyc
2.0
Citric Acid Cycle
SubPathway
Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
Guanidoacetic acid
70-26-8
CAS
ADENOSYL-HOMO-CYS
BioCyc
ReactionCatalysis41
ACTIVATION
6026
ChemSpider
1.0
SMILES
OC(=O)C1=NCCC1
2.1.3.3
false
2.1.3.3
Carbamoyl phosphate + Ornithine → Citrulline + Phosphate
LEFT_TO_RIGHT
C21H30N7O17P3
NADPH
745.0911
Mitochondrion
C21H28N7O17P3
NADP
743.07544
C5H12N2O2
Ornithine
132.08987
6.1.1.19
false
6.1.1.19
Adenosine triphosphate + L-Arginine + tRNA(Arg) → Adenosine monophosphate + L-Arginyl-tRNA(Arg) + Pyrophosphate
LEFT_TO_RIGHT
CHEBI:16919
ChEBI
O43272
UniProt
GLY
BioCyc
HMDB0000208
HMDB
1187-99-1
CAS
5810
PubChem-compound
579
ChemSpider
Water
HMDB0001301
HMDB
HMDB0000214
HMDB
SMILES
OC(=O)CCC(=O)C(O)=O
HMDB0000217
HMDB
GO:0005886
GENE ONTOLOGY
124-38-9
CAS
CHEBI:15414
ChEBI
NADP
C03079
KEGG Compound
1.0
567
ChemSpider
440851
PubChem-compound
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](OP(O)(O)=O)[C@@H]2O)N2C=NC3=C2N=CN=C3N)[C@@H](O)[C@H]1O
566
ChemSpider
1.0
HMDB0000221
HMDB
SMILES
NCCC[C@H](N)C(O)=O
5257127
PubChem-compound
HMDB0000223
HMDB
C5H7NO3
Pyrroline hydroxycarboxylic acid
129.04259
SMILES
NC(=O)C1=CN(C=CC1)[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OC[C@H]2O[C@H]([C@H](OP(O)(O)=O)[C@@H]2O)N2C=NC3=C2N=CN=C3N)[C@@H](O)[C@H]1O
7664-41-7
CAS
GLT
BioCyc
Oxygen
53-57-6
CAS
SMILES
OC(=O)[C@@H]1CCCN1
S-ADENOSYLMETHIONINE
BioCyc
222
PubChem-compound
Hydrogen Ion
SMILES
OP(O)(O)=O
1.0
H4O7P2
Pyrophosphate
177.94322
6082
ChemSpider
C4H6O4
Succinic acid
118.02661
Ornithine
Arginine and Proline Metabolism
C10H15N5O10P2
Adenosine diphosphate
427.02942
SMILES
N[C@@H](CC(O)=O)C(O)=O
Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
1.0
SMILES
N[C@@H](CCCNC(N)=O)C(O)=O
10.0
3.0
CPD-8587
BioCyc
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
CHEBI:15682
ChEBI
4-Hydroxyproline
Proline dehydrogenase 1, mitochondrial
1.0
Mitochondrial ornithine transporter 1
1-Pyrroline-4-hydroxy-2-carboxylate
CARBAMOYL-P
BioCyc
2.0
P14920
UniProt
Probable arginine--tRNA ligase, mitochondrial
Cytoplasm
1.0
1.0
CHEBI:15422
ChEBI
Glycine
1.0
Homo sapiens
8988
PubChem-compound
388299
ChemSpider
O2
Oxygen
31.98983
1.0
PYRROLINE-HYDROXY-CARBOXYLATE
BioCyc
Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
CREATINE-P
BioCyc
Water
CHEBI:16526
ChEBI
CHEBI:16761
ChEBI
CHEBI:15428
ChEBI
L-Proline
CHEBI:16338
ChEBI
145068
PubChem-compound
1059
PubChem-compound
Oxoglutaric acid
5893
PubChem-compound
1.14.11.2
false
1.14.11.2
L-Proline + Oxoglutaric acid + Oxygen → 4-Hydroxyproline + Carbon dioxide + Succinic acid
LEFT_TO_RIGHT
1.0
1.0
CHEBI:16344
ChEBI
278
PubChem-compound
CHEBI:16349
ChEBI
L-Prolyl-tRNA(Pro)
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
[N]=O
5886
PubChem-compound
1.0
CHEBI:17672
ChEBI
L-4-HYDROXY-PROLINE
BioCyc
280
PubChem-compound
2.0
1.0
P00966
UniProt
ReactionCatalysis136
ACTIVATION
ReactionCatalysis133
ACTIVATION
CHEBI:16313
ChEBI
D-Proline
SMILES
N[C@@H](CCSC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC2=C1N=CN=C2N)C(O)=O
4573749
ChemSpider
Succinic acid
1.0
3946848
PubChem-compound
1061
PubChem-compound
HMDB0001967
HMDB
8640
ChemSpider
24762165
PubChem-compound
1.0
tRNA(Pro)
L-ORNITHINE
BioCyc
S-Adenosylmethionine
P17174
UniProt