EC: 2.6.1.19

4-aminobutyrate-2-oxoglutarate transaminase (4-aminobutanoate:2-oxoglutarate aminotransferase)

enzyme lambda metabolic reaction experiment
uniprot:P80404 [4-aminobutyrate transaminase(Enzyme) wildtype]

4_Aminobutanoate + 2_Oxoglutarate -> L_Glutamate + Succinate_semialdehyde		 (( (Vmax * B) ) / (Km + B)) buffer: 0.3 M Tris-HCl
PH: 8.6
Temperature: 37
uniprot:P80404 [4-aminobutyrate transaminase(Enzyme) wildtype]

4_Aminobutanoate + 2_Oxoglutarate -> L_Glutamate + Succinate_semialdehyde		 (( (Vmax * A) ) / (Km + A)) buffer: 0.3 M Tris-HCl
PH: 8.6
Temperature: 37
uniprot:P50554 [4-aminobutyrate transaminase(Enzyme) wildtype]

4_Aminobutanoate + 2_Oxoglutarate -> Succinate_semialdehyde + L_Glutamate		 (( (Vmax * A) ) / (Km + A)) buffer: 0.3 M Tris-HCl
PH: 8.6
Temperature: 37
uniprot:P50554 [4-aminobutyrate transaminase(Enzyme) wildtype]

2_Oxoglutarate + 4_Aminobutanoate -> L_Glutamate + Succinate_semialdehyde		 (( (Vmax * B) ) / (Km + B)) buffer: 0.3 M Tris-HCl
PH: 8.6
Temperature: 37
uniprot:P50554 [4-Aminobutanoate]

2_Oxoglutarate + beta_Alanine -> 3_Oxopropanoate + L_Glutamate		 (1 / (( (( (( (( (Kd_A / Vmax) ) * (1 + (I / Kd_I))) ) * 1) ) / A) ) + (1 / Vmax))) buffer: 50 mM triethanolamine, 5 mM 2-mercaptoethanol, 0.5 mM pyridoxal 5'-phosphate
PH: 7.3
Temperature: 37
uniprot:P50554 [4-Aminobutanoate]

2_Oxoglutarate + beta_Alanine -> L_Glutamate + 3_Oxopropanoate		 (1 / (( (( (( (( (Kd_A / Vmax) ) * (1 + (I / Kd_I))) ) * 1) ) / A) ) + (1 / Vmax))) buffer: 50 mM triethanolamine, 5 mM 2-mercaptoethanol, 0.5 mM pyridoxal 5'-phosphate
PH: 7.3
Temperature: 37
uniprot:P50554 [L-3-Amino-isobutanoate]

2_Oxoglutarate + beta_Alanine -> L_Glutamate + 3_Oxopropanoate		 (( (Vmax * A) ) / (( (Km * (1 + (I / Ki))) ) + A)) buffer: 50 mM Sodium borate, 5 mM beta-Mercaptoethanol
PH: 8.8
Temperature: 37
uniprot:P50554 [Pyridoxal phosphate]

beta_Alanine + 2_Oxoglutarate -> 3_Oxopropanoate + L_Glutamate		 (( (Vmax * A) ) / (Km + A)) buffer: 50 mM Sodium borate, 5 mM beta-Mercaptoethanol
PH: 8.8
Temperature: 37
- [4-aminobutyrate transaminase(Enzyme) mutant E211S/I50H/V80T]

4_Aminobutanoate + 2_Oxoglutarate -> L_Glutamate + Succinate_semialdehyde		 (( (( (( (E * kcat) ) * A) ) * B) ) / (( (( (Ka * B) ) + (Kb * A)) ) + (A * B))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [4-aminobutyrate transaminase(Enzyme) mutant E211S]

4_Aminobutanoate + 2_Oxoglutarate -> Succinate_semialdehyde + L_Glutamate		 (( (( (( (E * kcat) ) * A) ) * B) ) / (( (( (Ka * B) ) + (Kb * A)) ) + (A * B))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [4-aminobutyrate transaminase(Enzyme) mutant V241A]

4_Aminobutanoate + 2_Oxoglutarate -> Succinate_semialdehyde + L_Glutamate		 (( (( (( (E * kcat) ) * A) ) * B) ) / (( (( (Ka * B) ) + (Kb * A)) ) + (A * B))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [4-aminobutyrate transaminase(Enzyme) mutant I50Q/G295Y]

2_Oxoglutarate + 4_Aminobutanoate -> L_Glutamate + Succinate_semialdehyde		 (( (( (( (E * kcat) ) * A) ) * B) ) / (( (( (Ka * B) ) + (Kb * A)) ) + (A * B))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [4-aminobutyrate transaminase(Enzyme) mutant E211S/I50G/C77K]

2_Oxoglutarate + 4_Aminobutanoate -> Succinate_semialdehyde + L_Glutamate		 (( (( (( (E * kcat) ) * A) ) * B) ) / (( (( (Ka * B) ) + (Kb * A)) ) + (A * B))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [4-aminobutyrate transaminase(Enzyme) wildtype]

2_Oxoglutarate + L_Aspartate -> Oxaloacetate + L_Glutamate		 (( (( (E * kcat) ) * A) ) / (Ka + A)) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [4-aminobutyrate transaminase(Enzyme) wildtype]

2_Oxoglutarate + L_Aspartate -> L_Glutamate + Oxaloacetate		 (( (( (E * kcat) ) * B) ) / (Kb + B)) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [4-aminobutyrate transaminase(Enzyme) mutant I50Q]

2_Oxoglutarate + 4_Aminobutanoate -> Succinate_semialdehyde + L_Glutamate		 (( (( (( (E * kcat) ) * A) ) * B) ) / (( (( (( (Ka * B) ) * (1 + (B / Ki_b))) ) + (( (Kb * A) ) * (1 + (A / Ki_a)))) ) + (A * B))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [4-aminobutyrate transaminase(Enzyme) wildtype]

4_Aminobutanoate + 2_Oxoglutarate -> Succinate_semialdehyde + L_Glutamate		 (( (( (( (E * kcat) ) * A) ) * B) ) / (( (( (( (Ka * B) ) * (1 + (B / Ki_b))) ) + (Kb * A)) ) + (A * B))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [4-aminobutyrate transaminase(Enzyme) mutant E211S]

L_Aspartate + 2_Oxoglutarate -> L_Glutamate + Oxaloacetate		 (( (( (E * kcat) ) * B) ) / (( (Kb + B) ) + (( (B ^ 2) ) / Ki_b))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [4-aminobutyrate transaminase(Enzyme) mutant E211S]

2_Oxoglutarate + L_Aspartate -> Oxaloacetate + L_Glutamate		 (( (( (E * kcat) ) * A) ) / (Ka + A)) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [Glutarate]

2_Oxoglutarate + 4_Aminobutanoate -> Succinate_semialdehyde + L_Glutamate		 (( (( (( (E * kcat) ) * A) ) * B) ) / (( (( (( (Ka * B) ) * (1 + (I / Ki))) ) + (Kb * A)) ) + (A * B))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [Glutarate]

4_Aminobutanoate + 2_Oxoglutarate -> Succinate_semialdehyde + L_Glutamate		 (( (( (( (E * kcat) ) * A) ) * B) ) / (( (( (( (Ka * B) ) * (1 + (I / Ki))) ) + (Kb * A)) ) + (A * B))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [Succinate]

4_Aminobutanoate + 2_Oxoglutarate -> L_Glutamate + Succinate_semialdehyde		 (( (( (( (E * kcat) ) * A) ) * B) ) / (( (( (( (Ka * B) ) * (1 + (I / Ki))) ) + (Kb * A)) ) + (A * B))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [Succinate]

2_Oxoglutarate + 4_Aminobutanoate -> Succinate_semialdehyde + L_Glutamate		 (( (( (( (E * kcat) ) * A) ) * B) ) / (( (( (( (Ka * B) ) * (1 + (I / Ki))) ) + (Kb * A)) ) + (A * B))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [Maleate]

4_Aminobutanoate + 2_Oxoglutarate -> Succinate_semialdehyde + L_Glutamate		 (( (( (( (E * kcat) ) * A) ) * B) ) / (( (( (( (Ka * B) ) * (1 + (I / Ki))) ) + (Kb * A)) ) + (A * B))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [Maleate]

2_Oxoglutarate + 4_Aminobutanoate -> Succinate_semialdehyde + L_Glutamate		 (( (( (( (E * kcat) ) * A) ) * B) ) / (( (( (( (Ka * B) ) * (1 + (I / Ki))) ) + (Kb * A)) ) + (A * B))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [Aminooxyacetate]

4_Aminobutanoate + 2_Oxoglutarate -> Succinate_semialdehyde + L_Glutamate		 (( (( (( (E * kcat) ) * A) ) * B) ) / (( (( (( (Ka * B) ) * (1 + (I / Ki))) ) + (Kb * A)) ) + (A * B))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [Glutarate]

4_Aminobutanoate + 2_Oxoglutarate -> Succinate_semialdehyde + L_Glutamate		 (( (( (( (E * kcat) ) * A) ) * B) ) / (( (( (( (Ka * B) ) * (1 + (I / Ki))) ) + (Kb * A)) ) + (A * B))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [Succinate]

4_Aminobutanoate + 2_Oxoglutarate -> L_Glutamate + Succinate_semialdehyde		 (( (( (( (E * kcat) ) * A) ) * B) ) / (( (( (( (Ka * B) ) * (1 + (I / Ki))) ) + (Kb * A)) ) + (A * B))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [Maleate]

2_Oxoglutarate + 4_Aminobutanoate -> L_Glutamate + Succinate_semialdehyde		 (( (( (( (E * kcat) ) * A) ) * B) ) / (( (( (( (Ka * B) ) * (1 + (I / Ki))) ) + (Kb * A)) ) + (A * B))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [Aminooxyacetate]

2_Oxoglutarate + 4_Aminobutanoate -> L_Glutamate + Succinate_semialdehyde		 (( (( (( (E * kcat) ) * A) ) * B) ) / (( (( (( (Ka * B) ) * (1 + (I / Ki))) ) + (Kb * A)) ) + (A * B))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
- [Aminooxyacetate]

4_Aminobutanoate + 2_Oxoglutarate -> Succinate_semialdehyde + L_Glutamate		 (( (( (( (E * kcat) ) * A) ) * B) ) / (( (( (( (Ka * B) ) * (1 + (I / Ki))) ) + (Kb * A)) ) + (A * B))) buffer: 100 mM TAE-HCl, 5 mM DTT, 1 mM EDTA, 100 µM pyridoxal 5'-phosphate, 100 mM KCl
PH: 7.8
Temperature: 25
uniprot:P80147 [4-aminobutyrate transaminase(Enzyme) wildtype]

2_Oxoglutarate + 4_Aminobutanoate -> Succinate_semialdehyde + L_Glutamate		 (( (( (Vmax * A) ) * B) ) / (( (( (Ka * B) ) + (Kb * A)) ) + (A * B))) buffer: 0.2 M Phosphate, 0.1 µM PLP, 0.03 µM glutathione
PH: 8
Temperature: 37
uniprot:P80147 [4-aminobutyrate transaminase(Enzyme) wildtype]

4_Aminobutanoate + 2_Oxoglutarate -> Succinate_semialdehyde + L_Glutamate		 (( (Vmax * A) ) / (Km + A)) buffer: 0.2 M Phosphate, 0.1 µM PLP, 0.03 µM glutathione
PH: 8
Temperature: 37
uniprot:P80147 [4-aminobutyrate transaminase(Enzyme) wildtype]

2_Oxoglutarate + 4_Aminobutanoate -> L_Glutamate + Succinate_semialdehyde		 (( (Vmax * B) ) / (Km + B)) buffer: 0.2 M Phosphate, 0.1 µM PLP, 0.03 µM glutathione
PH: 8
Temperature: 37

Pathways

pathway id name
BioCyc:HUMAN_PWY-6535 4-aminobutyrate degradation
BioCyc:META_PWY-6537 4-aminobutanoate degradation II
BioCyc:META_PWY-6535 4-aminobutanoate degradation I
BioCyc:ECO_GLUTORN-PWY L-ornithine biosynthesis I
BioCyc:ECO_PWY-6535 4-aminobutanoate degradation I
BioCyc:ECO_PWY-6537 4-aminobutanoate degradation II
BioCyc:YEAST_PWY-6536 4-aminobutyrate degradation
BioCyc:YEAST_GLUDEG-I-PWY-1 glutamate degradation to succinate
BioCyc:MTBH37RV_PWY-6537 4-aminobutanoate degradation II
BioCyc:MTBCDC1551_PWY-6535 4-aminobutanoate degradation I
BioCyc:ECOL316407_PWY-6535 4-aminobutyrate degradation
BioCyc:HUMAN_GLUDEG-I-PWY GABA shunt
BioCyc:META_PWY-6536 4-aminobutanoate degradation III
BioCyc:META_GLUDEG-I-PWY GABA shunt
BioCyc:META_VALDEG-PWY L-valine degradation I
BioCyc:ECO_ARG+POLYAMINE-SYN superpathway of arginine and polyamine biosynthesis
BioCyc:ECO_ARGSYN-PWY L-arginine biosynthesis I (via L-ornithine)
BioCyc:MTBCDC1551_PWY-6536 4-aminobutanoate degradation III
BioCyc:MOUSE_PWY-6535 4-aminobutyrate degradation I
BioCyc:ECOL316407_ARGDEG-PWY superpathway of arginine, putrescine, and 4-aminobutyrate degradation
BioCyc:ECOL316407_ORNARGDEG-PWY superpathway of arginine and ornithine degradation
BioCyc:HUMAN_VALDEG-PWY valine degradation
BioCyc:HUMAN_BETA-ALA-DEGRADATION-I-PWY β-alanine degradation
BioCyc:META_ARGSYN-PWY L-arginine biosynthesis I (via L-ornithine)
BioCyc:META_GLUTORN-PWY L-ornithine biosynthesis I
BioCyc:ECO_4AMINOBUTMETAB-PWY superpathway of 4-aminobutanoate degradation
BioCyc:ECO_ARGDEG-PWY superpathway of L-arginine, putrescine, and 4-aminobutanoate degradation
BioCyc:ECO_ORNARGDEG-PWY superpathway of L-arginine and L-ornithine degradation
BioCyc:MTBH37RV_PWY-6536 4-aminobutanoate degradation III
BioCyc:MOUSE_BETA-ALA-DEGRADATION-I-PWY β-alanine degradation I
BioCyc:MOUSE_VALDEG-PWY valine degradation I
BioCyc:MOUSE_GLUDEG-I-PWY glutamate degradation III (via 4-aminobutyrate)
BioCyc:BSUB_PWY-6536 4-aminobutanoate degradation III
BioCyc:ECOL316407_4AMINOBUTMETAB-PWY superpathway of 4-aminobutyrate degradation
BioCyc:META_ARG+POLYAMINE-SYN superpathway of arginine and polyamine biosynthesis
BioCyc:META_4AMINOBUTMETAB-PWY superpathway of 4-aminobutanoate degradation
BioCyc:META_ARGDEG-PWY superpathway of L-arginine, putrescine, and 4-aminobutanoate degradation
BioCyc:META_ORNARGDEG-PWY superpathway of L-arginine and L-ornithine degradation
BioCyc:MTBCDC1551_GLUDEG-I-PWY GABA shunt
BioCyc:MTBCDC1551_PWY-6537 4-aminobutanoate degradation II
BioCyc:MOUSE_PWY-6473 4-aminobutyrate degradation IV
BioCyc:MOUSE_PWY-4321 glutamate degradation IV
BioCyc:ECOL316407_PWY-6537 4-aminobutyrate degradation II
PathBank:SMP0000812 Arginine Metabolism
PathBank:SMP0000813 Ornithine Metabolism
PathBank:SMP0002080 4-Aminobutanoate Degradation I
Reactome:R-CEL-112315 Transmission across Chemical Synapses
Reactome:R-HSA-112315 Transmission across Chemical Synapses
Reactome:R-HSA-888590 GABA synthesis, release, reuptake and degradation
Reactome:R-MMU-916853 Degradation of GABA
Reactome:R-RNO-112316 Neuronal System
Reactome:R-SCE-112315 Transmission across Chemical Synapses
Reactome:R-SCE-888590 GABA synthesis, release, reuptake and degradation
Reactome:R-SPO-112316 Neuronal System
Reactome:R-SPO-916853 Degradation of GABA
Reactome:R-SSC-112315 Transmission across Chemical Synapses
Reactome:R-SSC-888590 GABA synthesis, release, reuptake and degradation
Reactome:R-CEL-112316 Neuronal System
Reactome:R-CEL-888590 GABA synthesis, release, reuptake and degradation
Reactome:R-DDI-112310 Neurotransmitter release cycle
Reactome:R-DDI-916853 Degradation of GABA
Reactome:R-HSA-916853 Degradation of GABA
Reactome:R-MMU-112310 Neurotransmitter release cycle
Reactome:R-RNO-112315 Transmission across Chemical Synapses
Reactome:R-RNO-888590 GABA synthesis, release, reuptake and degradation
Reactome:R-SCE-112316 Neuronal System
Reactome:R-SPO-888590 GABA synthesis, release, reuptake and degradation
Reactome:R-SSC-916853 Degradation of GABA
Reactome:R-CEL-112310 Neurotransmitter release cycle
Reactome:R-DDI-112316 Neuronal System
Reactome:R-HSA-112316 Neuronal System
Reactome:R-MMU-112315 Transmission across Chemical Synapses
Reactome:R-MMU-888590 GABA synthesis, release, reuptake and degradation
Reactome:R-RNO-112310 Neurotransmitter release cycle
Reactome:R-SCE-112310 Neurotransmitter release cycle
Reactome:R-SCE-916853 Degradation of GABA
Reactome:R-SPO-112315 Transmission across Chemical Synapses
Reactome:R-SSC-112316 Neuronal System
Reactome:R-CEL-916853 Degradation of GABA
Reactome:R-DDI-112315 Transmission across Chemical Synapses
Reactome:R-DDI-888590 GABA synthesis, release, reuptake and degradation
Reactome:R-HSA-112310 Neurotransmitter release cycle
Reactome:R-MMU-112316 Neuronal System
Reactome:R-RNO-916853 Degradation of GABA
Reactome:R-SPO-112310 Neurotransmitter release cycle
Reactome:R-SSC-112310 Neurotransmitter release cycle
WikiPathways:WP4159 GABA receptor signaling
WikiPathways:WP4225 Pyrimidine metabolism and related diseases
WikiPathways:WP4157 GABA metabolism (aka GHB)
PathBank:SMP0120662 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I
PathBank:SMP0120666 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0120698 GABA-Transaminase Deficiency
PathBank:SMP0120714 Homocarnosinosis
PathBank:SMP0120722 Hypoacetylaspartia
PathBank:SMP0120734 Malonic Aciduria
PathBank:SMP0120778 Carnosinuria, Carnosinemia
PathBank:SMP0120786 Malonyl-CoA Decarboxylase Deficiency
PathBank:SMP0120806 3-Hydroxyisobutyric Aciduria
PathBank:SMP0120850 Succinic Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0120440 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency
PathBank:SMP0120444 3-Methylglutaconic Aciduria Type IV
PathBank:SMP0120496 Hyperinsulinism-Hyperammonemia Syndrome
PathBank:SMP0120516 Maple Syrup Urine Disease
PathBank:SMP0120528 Methylmalonic Aciduria Due to Cobalamin-Related Disorders
PathBank:SMP0120589 Isovaleric Acidemia
PathBank:SMP0000032 Valine, Leucine, and Isoleucine Degradation
PathBank:SMP0000016 Propanoate Metabolism
PathBank:SMP0000173 beta-Ketothiolase Deficiency
PathBank:SMP0000199 Maple Syrup Urine Disease
PathBank:SMP0000384 Methylmalonate Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0000339 Hyperinsulinism-Hyperammonemia Syndrome
PathBank:SMP0000141 3-Methylglutaconic Aciduria Type IV
PathBank:SMP0000492 Ureidopropionase Deficiency
PathBank:SMP0000524 Isovaleric Acidemia
PathBank:SMP0087165 Aspartate Metabolism
PathBank:SMP0087169 Glutamate Metabolism
PathBank:SMP0087234 Valine, Leucine, and Isoleucine Degradation
PathBank:SMP0087278 Glutamate Metabolism
PathBank:SMP0087426 Aspartate Metabolism
PathBank:SMP0063656 Propanoate Metabolism
PathBank:SMP0120661 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency
PathBank:SMP0120665 3-Methylglutaconic Aciduria Type IV
PathBank:SMP0120681 Canavan Disease
PathBank:SMP0120745 Methylmalonic Aciduria
PathBank:SMP0120757 Propionic Acidemia
PathBank:SMP0120777 Ureidopropionase Deficiency
PathBank:SMP0120805 3-Hydroxyisobutyric Acid Dehydrogenase Deficiency
PathBank:SMP0120439 2-Methyl-3-hydroxybutryl-CoA Dehydrogenase Deficiency
PathBank:SMP0120443 3-Methylglutaconic Aciduria Type III
PathBank:SMP0120459 beta-Ketothiolase Deficiency
PathBank:SMP0120503 Isovaleric Aciduria
PathBank:SMP0120523 Methylmalonate Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0120588 Isobutyryl-CoA Dehydrogenase Deficiency
PathBank:SMP0000138 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency
PathBank:SMP0000140 3-Methylglutaconic Aciduria Type III
PathBank:SMP0000385 Homocarnosinosis
PathBank:SMP0000238 Isovaleric Aciduria
PathBank:SMP0000201 Methylmalonic Aciduria Due to Cobalamin-Related Disorders
PathBank:SMP0000136 2-Hydroxyglutric Aciduria (D and L Form)
PathBank:SMP0000523 Isobutyryl-CoA Dehydrogenase Deficiency
PathBank:SMP0000567 Succinic Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0087180 beta-Alanine Metabolism
PathBank:SMP0087341 Propanoate Metabolism
PathBank:SMP0063599 beta-Alanine Metabolism
PathBank:SMP0063689 Valine, Leucine, and Isoleucine Degradation
PathBank:SMP0120660 2-Methyl-3-hydroxybutryl-CoA Dehydrogenase Deficiency
PathBank:SMP0120664 3-Methylglutaconic Aciduria Type III
PathBank:SMP0120716 Hyperinsulinism-Hyperammonemia Syndrome
PathBank:SMP0120736 Maple Syrup Urine Disease
PathBank:SMP0120748 Methylmalonic Aciduria Due to Cobalamin-Related Disorders
PathBank:SMP0120808 Isovaleric Acidemia
PathBank:SMP0120441 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I
PathBank:SMP0120445 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0120461 Canavan Disease
PathBank:SMP0120525 Methylmalonic Aciduria
PathBank:SMP0120537 Propionic Acidemia
PathBank:SMP0120558 Carnosinuria, Carnosinemia
PathBank:SMP0120586 3-Hydroxyisobutyric Acid Dehydrogenase Deficiency
PathBank:SMP0000067 Aspartate Metabolism
PathBank:SMP0000137 2-Methyl-3-hydroxybutyryl-CoA Dehydrogenase Deficiency
PathBank:SMP0000237 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I
PathBank:SMP0000200 Methylmalonic Aciduria
PathBank:SMP0000175 Canavan Disease
PathBank:SMP0000351 GABA-Transaminase Deficiency
PathBank:SMP0000493 Carnosinuria, Carnosinemia
PathBank:SMP0000521 3-Hydroxyisobutyric Acid Dehydrogenase Deficiency
PathBank:SMP0002303 Glutamate Metabolism
PathBank:SMP0087248 Propanoate Metabolism
PathBank:SMP0087276 Aspartate Metabolism
PathBank:SMP0087284 beta-Alanine Metabolism
PathBank:SMP0087428 Glutamate Metabolism
PathBank:SMP0087432 beta-Alanine Metabolism
PathBank:SMP0087480 Propanoate Metabolism
PathBank:SMP0063590 Glutamate Metabolism
PathBank:SMP0120659 2-Hydroxyglutric Aciduria (D and L Form)
PathBank:SMP0120663 3-Methylglutaconic Aciduria Type I
PathBank:SMP0120679 beta-Ketothiolase Deficiency
PathBank:SMP0120723 Isovaleric Aciduria
PathBank:SMP0120743 Methylmalonate Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0120807 Isobutyryl-CoA Dehydrogenase Deficiency
PathBank:SMP0120438 2-Hydroxyglutric Aciduria (D and L Form)
PathBank:SMP0120442 3-Methylglutaconic Aciduria Type I
PathBank:SMP0120478 GABA-Transaminase Deficiency
PathBank:SMP0120494 Homocarnosinosis
PathBank:SMP0120502 Hypoacetylaspartia
PathBank:SMP0120514 Malonic Aciduria
PathBank:SMP0120567 Malonyl-CoA Decarboxylase Deficiency
PathBank:SMP0120587 3-Hydroxyisobutyric Aciduria
PathBank:SMP0120631 Succinic Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0000007 beta-Alanine Metabolism
PathBank:SMP0000072 Glutamate Metabolism
PathBank:SMP0000236 Propionic Acidemia
PathBank:SMP0000139 3-Methylglutaconic Aciduria Type I
PathBank:SMP0000243 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency
PathBank:SMP0000192 Hypoacetylaspartia
PathBank:SMP0000198 Malonic Aciduria
PathBank:SMP0000502 Malonyl-CoA Decarboxylase Deficiency
PathBank:SMP0000522 3-Hydroxyisobutyric Aciduria
PathBank:SMP0087327 Valine, Leucine, and Isoleucine Degradation
PathBank:SMP0063597 Aspartate Metabolism