EC: 1.2.4.4

3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) (3-methyl-2-oxobutanoate:[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]-lipoyllysine 2-oxidoreductase (decarboxylating, acceptor-2-methylpropanoylating))

enzyme lambda metabolic reaction experiment
- [Thiamine diphosphate]

NAD+ + Pyruvate + Coenzyme_A -> CO2 + H+ + NADH + Acetyl_CoA		 (( (Vmax * S) ) / (Km + S)) buffer: 30 mM potassium phosphate, 5 mM DTT, 0.2 mM MgCl2, 100 µg/ml BSA, 20-30 µg/ml human liver lipoamide oxidoreductase
PH: 7.2
Temperature: 37
- [Thiamine diphosphate]

Coenzyme_A + NAD+ + 2_Oxoglutarate -> CO2 + NADH + Succinyl_CoA + H+		 (( (Vmax * S) ) / (Km + S)) buffer: 30 mM potassium phosphate, 5 mM DTT, 0.2 mM MgCl2, 100 µg/ml BSA, 20-30 µg/ml human liver lipoamide oxidoreductase
PH: 7.2
Temperature: 37
- [Thiamine diphosphate]

Coenzyme_A + 3_Methyl_2_oxopentanoate + NAD+ -> H+ + CO2 + 2_Methylbutanoyl_CoA + NADH		 (( (Vmax * S) ) / (Km + S)) buffer: 30 mM potassium phosphate, 5 mM DTT, 0.2 mM MgCl2, 100 µg/ml BSA, 20-30 µg/ml human liver lipoamide oxidoreductase
PH: 7.2
Temperature: 37
- [Thiamine diphosphate]

Coenzyme_A + NAD+ + alpha_Ketovaline -> NADH + CO2 + H+ + 2_Methylpropanoyl_CoA		 (( (Vmax * S) ) / (Km + S)) buffer: 30 mM potassium phosphate, 5 mM DTT, 0.2 mM MgCl2, 100 µg/ml BSA, 20-30 µg/ml human liver lipoamide oxidoreductase
PH: 7.2
Temperature: 37
- [Thiamine diphosphate]

NAD+ + 4_Methyl_2_oxopentanoate + Coenzyme_A -> NADH + CO2 + 3_Methylbutanoyl_CoA + H+		 (( (Vmax * S) ) / (Km + S)) buffer: 30 mM potassium phosphate, 5 mM DTT, 0.2 mM MgCl2, 100 µg/ml BSA, 20-30 µg/ml human liver lipoamide oxidoreductase
PH: 7.2
Temperature: 37
uniprot:P11960 [Thiamine diphosphate]

NAD+ + Coenzyme_A + Pyruvate -> H+ + NADH + CO2 + Acetyl_CoA		 (( (Vmax * A) ) / (Km + A)) buffer: 30 mM Potassium phosphate, 0.2 mM MgCl2, 5 mM Dithiothreitol, 100 µg/ml Bovine serum albumin
PH: 7.2
Temperature: 37
uniprot:P11960 [Thiamine diphosphate]

Coenzyme_A + 2_Oxoglutarate + NAD+ -> H+ + NADH + Succinyl_CoA + CO2		 (( (Vmax * A) ) / (Km + A)) buffer: 30 mM Potassium phosphate, 0.2 mM MgCl2, 5 mM Dithiothreitol, 100 µg/ml Bovine serum albumin
PH: 7.2
Temperature: 37
uniprot:P11960 [Thiamine diphosphate]

3_Methyl_2_oxopentanoate + Coenzyme_A + NAD+ -> 2_Methylbutanoyl_CoA + NADH + H+ + CO2		 (( (Vmax * A) ) / (Km + A)) buffer: 30 mM Potassium phosphate, 0.2 mM MgCl2, 5 mM Dithiothreitol, 100 µg/ml Bovine serum albumin
PH: 7.2
Temperature: 37
uniprot:P11960 [Thiamine diphosphate]

NAD+ + Coenzyme_A + alpha_Ketovaline -> 2_Methylpropanoyl_CoA + NADH + CO2 + H+		 (( (Vmax * A) ) / (Km + A)) buffer: 30 mM Potassium phosphate, 0.2 mM MgCl2, 5 mM Dithiothreitol, 100 µg/ml Bovine serum albumin
PH: 7.2
Temperature: 37
uniprot:P11960 [Thiamine diphosphate]

Coenzyme_A + 4_Methyl_2_oxopentanoate + NAD+ -> NADH + CO2 + 3_Methylbutanoyl_CoA + H+		 (( (Vmax * A) ) / (Km + A)) buffer: 30 mM Potassium phosphate, 0.2 mM MgCl2, 5 mM Dithiothreitol, 100 µg/ml Bovine serum albumin
PH: 7.2
Temperature: 37
uniprot:P21953 [Thiamine diphosphate]

alpha_Ketovaline + Coenzyme_A + NAD+ -> NADH + 2_Methylpropanoyl_CoA + H+ + CO2		 (( (( (E * kcat) ) * S) ) / (Km + S)) buffer: 30 mM phosphate, 100 mM NaCl, 2 mM MgCl2, 2 mM dithiothreitol, 0.1 % Triton X-100
PH: 7.5
Temperature: 30
uniprot:P21953 [Thiamine diphosphate]

Acceptor + alpha_Ketovaline + H2O -> Isobutanoate + Reduced_acceptor + CO2 + H+		 (( (( (E * kcat) ) * S) ) / (Km + S)) buffer: 100 mM potassium phosphate, 2.0 mM MgCl2
PH: 7.5
Temperature: 30
uniprot:P21953 [Thiamine diphosphate]

alpha_Ketovaline + Coenzyme_A + NAD+ -> NADH + H+ + 2_Methylpropanoyl_CoA + CO2		 (( (( (E * kcat) ) * S) ) / (Km + S)) buffer: 30 mM phosphate, 100 mM NaCl, 2 mM MgCl2, 2 mM dithiothreitol, 0.1 % Triton X-100
PH: 7.5
Temperature: 30
uniprot:P21953 [Thiamine diphosphate]

alpha_Ketovaline + Coenzyme_A + NAD+ -> CO2 + H+ + 2_Methylpropanoyl_CoA + NADH		 (( (( (E * kcat) ) * S) ) / (Km + S)) buffer: 30 mM phosphate, 100 mM NaCl, 2 mM MgCl2, 2 mM dithiothreitol, 0.1 % Triton X-100
PH: 7.5
Temperature: 30
uniprot:P21953 [Thiamine diphosphate]

Coenzyme_A + alpha_Ketovaline + NAD+ -> 2_Methylpropanoyl_CoA + H+ + NADH + CO2		 (( (( (E * kcat) ) * S) ) / (Km + S)) buffer: 30 mM phosphate, 100 mM NaCl, 2 mM MgCl2, 2 mM dithiothreitol, 0.1 % Triton X-100
PH: 7.5
Temperature: 30
uniprot:P21953 [Thiamine diphosphate]

alpha_Ketovaline + Acceptor + H2O -> Reduced_acceptor + H+ + Isobutanoate + CO2		 (( (( (E * kcat) ) * S) ) / (Km + S)) buffer: 100 mM potassium phosphate, 2.0 mM MgCl2
PH: 7.5
Temperature: 30
uniprot:P21953 [Thiamine diphosphate]

alpha_Ketovaline + Acceptor + H2O -> CO2 + Isobutanoate + Reduced_acceptor + H+		 (( (( (E * kcat) ) * S) ) / (Km + S)) buffer: 100 mM potassium phosphate, 2.0 mM MgCl2
PH: 7.5
Temperature: 30
uniprot:P21953 [Thiamine diphosphate]

alpha_Ketovaline + H2O + Acceptor -> Reduced_acceptor + H+ + Isobutanoate + CO2		 (( (( (E * kcat) ) * S) ) / (Km + S)) buffer: 100 mM potassium phosphate, 2.0 mM MgCl2
PH: 7.5
Temperature: 30
uniprot:P21953 [Thiamine diphosphate]

alpha_Ketovaline + H2O + Acceptor -> Isobutanoate + CO2 + Reduced_acceptor + H+		 (( (( (E * kcat) ) * S) ) / (Km + S)) buffer: 100 mM potassium phosphate, 2.0 mM MgCl2
PH: 7.5
Temperature: 30
uniprot:P21953 [Thiamine diphosphate]

H2O + Acceptor + alpha_Ketovaline -> Reduced_acceptor + H+ + CO2 + Isobutanoate		 (( (( (E * kcat) ) * S) ) / (Km + S)) buffer: 100 mM potassium phosphate, 2.0 mM MgCl2
PH: 7.5
Temperature: 30
- [Thiamine diphosphate]

Coenzyme_A + NAD+ + alpha_Ketovaline -> H+ + NADH + 2_Methylpropanoyl_CoA + CO2		 (( (Vmax * A) ) / (Km_A + A)) buffer: *
PH: 7
Temperature: 36
- [Thiamine diphosphate]

alpha_Ketovaline + NAD+ + Coenzyme_A -> H+ + 2_Methylpropanoyl_CoA + NADH + CO2		 (( (Vmax * A) ) / (Km_A + A)) buffer: *
PH: 7
Temperature: 36
- [Thiamine diphosphate]

alpha_Ketovaline + Coenzyme_A + NAD+ -> CO2 + 2_Methylpropanoyl_CoA + H+ + NADH		 (( (Vmax * A) ) / (Km_A + A)) buffer: *
PH: 7
Temperature: 36
- [Thiamine diphosphate]

Coenzyme_A + alpha_Ketovaline + NAD+ -> NADH + CO2 + H+ + 2_Methylpropanoyl_CoA		 (( (Vmax * S) ) / (Km + S)) buffer: *
PH: 7
Temperature: 36
- [Thiamine diphosphate]

Coenzyme_A + NAD+ + alpha_Ketovaline -> H+ + CO2 + NADH + 2_Methylpropanoyl_CoA		 (( (Vmax * S) ) / (Km + S)) buffer: *
PH: 7
Temperature: 36
- [Thiamine diphosphate]

alpha_Ketovaline + NAD+ + Coenzyme_A -> NADH + H+ + CO2 + 2_Methylpropanoyl_CoA		 (( (Vmax * S) ) / (Km + S)) buffer: *
PH: 7
Temperature: 36
- [Thiamine diphosphate]

alpha_Ketovaline + Coenzyme_A + NAD+ -> CO2 + 2_Methylpropanoyl_CoA + NADH + H+		 (( (Vmax * S) ) / (Km + S)) buffer: *
PH: 7
Temperature: 36
- [Thiamine diphosphate]

Coenzyme_A + NAD+ + alpha_Ketovaline -> NADH + CO2 + H+ + 2_Methylpropanoyl_CoA		 (( (Vmax * S) ) / (Km + S)) buffer: *
PH: 7
Temperature: 36
- [Thiamine diphosphate]

NAD+ + alpha_Ketovaline + Coenzyme_A -> H+ + CO2 + 2_Methylpropanoyl_CoA + NADH		 (( (Vmax * S) ) / (Km + S)) buffer: *
PH: 7
Temperature: 36
- [Thiamine diphosphate]

NAD+ + Coenzyme_A + alpha_Ketovaline -> H+ + CO2 + 2_Methylpropanoyl_CoA + NADH		 (( (Vmax * A) ) / (Km_A + A)) buffer: *
PH: 7
Temperature: 36
- [Thiamine diphosphate]

alpha_Ketovaline + Coenzyme_A + NAD+ -> H+ + 2_Methylpropanoyl_CoA + CO2 + NADH		 (( (Vmax * A) ) / (Km_A + A)) buffer: *
PH: 7
Temperature: 36
- [Thiamine diphosphate]

alpha_Ketovaline + Coenzyme_A + NAD+ -> H+ + NADH + CO2 + 2_Methylpropanoyl_CoA		 (( (Vmax * A) ) / (Km_A + A)) buffer: *
PH: 7
Temperature: 36
- [Thiamine diphosphate]

Coenzyme_A + NAD+ + alpha_Ketovaline -> 2_Methylpropanoyl_CoA + NADH + CO2 + H+		 (( (Vmax * A) ) / (Km_A + A)) buffer: *
PH: 7
Temperature: 36
- [Thiamine diphosphate]

alpha_Ketovaline + NAD+ + Coenzyme_A -> 2_Methylpropanoyl_CoA + CO2 + H+ + NADH		 (( (Vmax * A) ) / (Km_A + A)) buffer: *
PH: 7
Temperature: 36
- [Thiamine diphosphate]

NAD+ + Coenzyme_A + alpha_Ketovaline -> 2_Methylpropanoyl_CoA + CO2 + NADH + H+		 (( (Vmax * A) ) / (Km_A + A)) buffer: *
PH: 7
Temperature: 36
- [Thiamine diphosphate]

4_Methyl_2_oxopentanoate + Coenzyme_A + NAD+ -> 3_Methylbutanoyl_CoA + NADH + CO2 + H+		 (( (Vmax * S) ) / (Km + S)) buffer: *
PH: 7
Temperature: 36
- [Thiamine diphosphate]

4_Methyl_2_oxopentanoate + Coenzyme_A + NAD+ -> 3_Methylbutanoyl_CoA + H+ + CO2 + NADH		 (( (Vmax * S) ) / (Km + S)) buffer: *
PH: 7
Temperature: 36
- [Thiamine diphosphate]

alpha_Ketovaline + Coenzyme_A + NAD+ -> H+ + 2_Methylpropanoyl_CoA + NADH + CO2		 (( (Vmax * S) ) / (Km + S)) buffer: *
PH: 7
Temperature: 36
- [Thiamine diphosphate]

NAD+ + Coenzyme_A + alpha_Ketovaline -> CO2 + NADH + H+ + 2_Methylpropanoyl_CoA		 (( (Vmax * S) ) / (Km + S)) buffer: *
PH: 7
Temperature: 36
- [Thiamine diphosphate]

alpha_Ketovaline + NAD+ + Coenzyme_A -> NADH + H+ + 2_Methylpropanoyl_CoA + CO2		 (( (Vmax * S) ) / (Km + S)) buffer: *
PH: 7
Temperature: 36
- [Mg2+]

Coenzyme_A + alpha_Ketovaline + NAD+ -> NADH + 2_Methylpropanoyl_CoA + H+ + CO2		 (( (Vmax * A) ) / (Km + A)) buffer: 30 mM Potassium phosphate, 4 µg/ml Rotenone
PH: 7.5
Temperature: 30
- [Mg2+]

Coenzyme_A + 4_Methyl_2_oxopentanoate + NAD+ -> NADH + H+ + 3_Methylbutanoyl_CoA + CO2		 (( (Vmax * A) ) / (Km + A)) buffer: 30 mM Potassium phosphate, 4 µg/ml Rotenone
PH: 7.5
Temperature: 30
- [Mg2+]

3_Methyl_2_oxopentanoate + Coenzyme_A + NAD+ -> NADH + H+ + 2_Methylbutanoyl_CoA + CO2		 (( (Vmax * A) ) / (Km + A)) buffer: 30 mM Potassium phosphate, 4 µg/ml Rotenone
PH: 7.5
Temperature: 30
- [Thiamine diphosphate]

NAD+ + Coenzyme_A + Pyruvate -> CO2 + NADH + Acetyl_CoA + H+		 (( (Vmax * A) ) / (Km + A)) buffer: 30 mM Potassium phosphate, 2 mM MgCl2, 1 mM Dithiothreitol, 0.1% Triton X-100, 10 units/ml Porcine heart dihydrolipoamide reductase
PH: 7.5
Temperature: 30
- [Thiamine diphosphate]

NAD+ + 3_Methyl_2_oxopentanoate + Coenzyme_A -> CO2 + H+ + NADH + 2_Methylbutanoyl_CoA		 (( (Vmax * A) ) / (Km + A)) buffer: 30 mM Potassium phosphate, 2 mM MgCl2, 1 mM Dithiothreitol, 0.1% Triton X-100, 10 units/ml Porcine heart dihydrolipoamide reductase
PH: 7.5
Temperature: 30
- [Thiamine diphosphate]

alpha_Ketovaline + Coenzyme_A + NAD+ -> NADH + H+ + 2_Methylpropanoyl_CoA + CO2		 (( (Vmax * A) ) / (Km + A)) buffer: 30 mM Potassium phosphate, 2 mM MgCl2, 1 mM Dithiothreitol, 0.1% Triton X-100, 10 units/ml Porcine heart dihydrolipoamide reductase
PH: 7.5
Temperature: 30
- [Thiamine diphosphate]

NAD+ + Coenzyme_A + 4_Methyl_2_oxopentanoate -> NADH + H+ + 3_Methylbutanoyl_CoA + CO2		 (( (Vmax * A) ) / (Km + A)) buffer: 30 mM Potassium phosphate, 2 mM MgCl2, 1 mM Dithiothreitol, 0.1% Triton X-100, 10 units/ml Porcine heart dihydrolipoamide reductase
PH: 7.5
Temperature: 30

Pathways

pathway id name
BioCyc:HUMAN_ILEUDEG-PWY isoleucine degradation
BioCyc:HUMAN_PWY-5328 superpathway of methionine degradation
BioCyc:META_ILEUDEG-PWY L-isoleucine degradation I
BioCyc:META_PWY-5328 superpathway of L-methionine salvage and degradation
BioCyc:ARA_VALDEG-PWY L-valine degradation I
BioCyc:MTBH37RV_PWY-5046 2-oxoisovalerate decarboxylation to isobutanoyl-CoA
BioCyc:MTBCDC1551_PWY-5046 2-oxoisovalerate decarboxylation to isobutanoyl-CoA
BioCyc:HUMAN_PWY-5046 2-oxoisovalerate decarboxylation to isobutanoyl-CoA
BioCyc:HUMAN_LEU-DEG2-PWY leucine degradation
BioCyc:META_PWY-5046 2-oxoisovalerate decarboxylation to isobutanoyl-CoA
BioCyc:META_PWY-5130 2-oxobutanoate degradation I
BioCyc:META_PWY66-428 L-threonine degradation V
BioCyc:META_VALDEG-PWY L-valine degradation I
BioCyc:ARA_LEU-DEG2-PWY L-leucine degradation I
BioCyc:ARA_ILEUDEG-PWY L-isoleucine degradation I
BioCyc:MOUSE_PWY3DJ-0 isoleucine degradation
BioCyc:MOUSE_PWY3DJ-6 Leucine Catabolism
BioCyc:HUMAN_PWY66-428 threonine degradation
BioCyc:HUMAN_VALDEG-PWY valine degradation
BioCyc:MTBCDC1551_VALDEG-PWY L-valine degradation I
BioCyc:MOUSE_PWY-5046 branched-chain α-keto acid dehydrogenase complex
BioCyc:HUMAN_PWY-5130 2-oxobutanoate degradation
BioCyc:META_LEU-DEG2-PWY L-leucine degradation I
BioCyc:ARA_PWY-5046 2-oxoisovalerate decarboxylation to isobutanoyl-CoA
BioCyc:MTBH37RV_VALDEG-PWY L-valine degradation I
BioCyc:MOUSE_PWY3DJ-86 valine degradation
BioCyc:BSUB_PWY-5046 2-oxoisovalerate decarboxylation to isobutanoyl-CoA
PlantCyc:ARA_VALDEG-PWY L-valine degradation I
PlantCyc:PLANT_ILEUDEG-PWY L-isoleucine degradation I
PlantCyc:PLANT_VALDEG-PWY L-valine degradation I
PlantCyc:ARA_ILEUDEG-PWY L-isoleucine degradation I
PlantCyc:ARA_LEU-DEG2-PWY L-leucine degradation I
PlantCyc:PLANT_LEU-DEG2-PWY L-leucine degradation I
PlantCyc:ARA_PWY-5046 2-oxoisovalerate decarboxylation to isobutanoyl-CoA
Reactome:R-HSA-9865118 Diseases of branched-chain amino acid catabolism
Reactome:R-HSA-9907570 Loss-of-function mutations in DLD cause MSUD3/DLDD
Reactome:R-BTA-9859138 BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV
Reactome:R-CEL-9859138 BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV
Reactome:R-HSA-9865113 Loss-of-function mutations in DBT cause MSUD2
Reactome:R-HSA-9859138 BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV
Reactome:R-HSA-9865114 Maple Syrup Urine Disease
Reactome:R-HSA-9912529 H139Hfs13* PPM1K causes a mild variant of MSUD
Reactome:R-DDI-9859138 BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV
Reactome:R-HSA-9865125 Loss-of-function mutations in BCKDHA or BCKDHB cause MSUD
Reactome:R-HSA-9912481 Branched-chain ketoacid dehydrogenase kinase deficiency
Reactome:R-MMU-9859138 BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV
Reactome:R-RNO-9859138 BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV
Reactome:R-BTA-1430728 Metabolism
Reactome:R-BTA-71291 Amino acid and derivative metabolism
Reactome:R-CEL-70895 Branched-chain amino acid catabolism
Reactome:R-HSA-70895 Branched-chain amino acid catabolism
Reactome:R-HSA-1643685 Disease
Reactome:R-MMU-1430728 Metabolism
Reactome:R-MMU-70895 Branched-chain amino acid catabolism
Reactome:R-RNO-70895 Branched-chain amino acid catabolism
Reactome:R-BTA-70895 Branched-chain amino acid catabolism
Reactome:R-DDI-71291 Amino acid and derivative metabolism
Reactome:R-CEL-71291 Amino acid and derivative metabolism
Reactome:R-HSA-71291 Amino acid and derivative metabolism
Reactome:R-HSA-5668914 Diseases of metabolism
Reactome:R-MMU-71291 Amino acid and derivative metabolism
Reactome:R-RNO-1430728 Metabolism
Reactome:R-RNO-71291 Amino acid and derivative metabolism
Reactome:R-CEL-1430728 Metabolism
Reactome:R-DDI-1430728 Metabolism
Reactome:R-DDI-70895 Branched-chain amino acid catabolism
Reactome:R-HSA-1430728 Metabolism
WikiPathways:WP4686 Leucine, isoleucine and valine metabolism
WikiPathways:WP5220 Metabolic reprogramming in pancreatic cancer
PathBank:SMP0120662 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I
PathBank:SMP0120734 Malonic Aciduria
PathBank:SMP0120786 Malonyl-CoA Decarboxylase Deficiency
PathBank:SMP0120806 3-Hydroxyisobutyric Aciduria
PathBank:SMP0120613 2-Ketoglutarate Dehydrogenase Complex Deficiency
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:SMP0000141 3-Methylglutaconic Aciduria Type IV
PathBank:SMP0000524 Isovaleric Acidemia
PathBank:SMP0002433 Leucine Degradation
PathBank:SMP0087234 Valine, Leucine, and Isoleucine Degradation
PathBank:SMP0087262 Threonine and 2-Oxobutanoate Degradation
PathBank:SMP0063661 Pyruvate Metabolism
PathBank:SMP0120661 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency
PathBank:SMP0120665 3-Methylglutaconic Aciduria Type IV
PathBank:SMP0120745 Methylmalonic Aciduria
PathBank:SMP0120757 Propionic Acidemia
PathBank:SMP0120805 3-Hydroxyisobutyric Acid Dehydrogenase Deficiency
PathBank:SMP0108760 Glutaminolysis and Cancer
PathBank:SMP0120531 Pyruvate Dehydrogenase Complex Deficiency
PathBank:SMP0120612 Mitochondrial Complex II Deficiency
PathBank:SMP0000138 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency
PathBank:SMP0000140 3-Methylglutaconic Aciduria Type III
PathBank:SMP0000238 Isovaleric Aciduria
PathBank:SMP0000201 Methylmalonic Aciduria Due to Cobalamin-Related Disorders
PathBank:SMP0000523 Isobutyryl-CoA Dehydrogenase Deficiency
PathBank:SMP0087341 Propanoate Metabolism
PathBank:SMP0120660 2-Methyl-3-hydroxybutryl-CoA Dehydrogenase Deficiency
PathBank:SMP0120664 3-Methylglutaconic Aciduria Type III
PathBank:SMP0120736 Maple Syrup Urine Disease
PathBank:SMP0120748 Methylmalonic Aciduria Due to Cobalamin-Related Disorders
PathBank:SMP0120808 Isovaleric Acidemia
PathBank:SMP0108758 The Oncogenic Action of 2-Hydroxyglutarate
PathBank:SMP0120533 Pyruvate Decarboxylase E1 Component Deficiency (PDHE1 Deficiency)
PathBank:SMP0120610 Congenital Lactic Acidosis
PathBank:SMP0120614 Pyruvate Dehydrogenase Deficiency (E3)
PathBank:SMP0120622 Primary Hyperoxaluria II, PH2
PathBank:SMP0000137 2-Methyl-3-hydroxybutyryl-CoA Dehydrogenase Deficiency
PathBank:SMP0000237 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I
PathBank:SMP0000200 Methylmalonic Aciduria
PathBank:SMP0000521 3-Hydroxyisobutyric Acid Dehydrogenase Deficiency
PathBank:SMP0002460 Valine Degradation
PathBank:SMP0087248 Propanoate Metabolism
PathBank:SMP0087480 Propanoate Metabolism
PathBank:SMP0087516 Threonine and 2-Oxobutanoate Degradation
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:SMP0120506 Leigh Syndrome
PathBank:SMP0120611 Fumarase Deficiency
PathBank:SMP0120615 Pyruvate Dehydrogenase Deficiency (E2)
PathBank:SMP0120623 Pyruvate Kinase Deficiency
PathBank:SMP0000452 Threonine and 2-Oxobutanoate Degradation
PathBank:SMP0000236 Propionic Acidemia
PathBank:SMP0000139 3-Methylglutaconic Aciduria Type I
PathBank:SMP0000198 Malonic Aciduria
PathBank:SMP0000502 Malonyl-CoA Decarboxylase Deficiency
PathBank:SMP0000522 3-Hydroxyisobutyric Aciduria
PathBank:SMP0002431 Isoleucine Degradation
PathBank:SMP0086930 Warburg Effect
PathBank:SMP0087327 Valine, Leucine, and Isoleucine Degradation
PathBank:SMP0087355 Threonine and 2-Oxobutanoate Degradation
PathBank:SMP0063477 Citric Acid Cycle
PathBank:SMP0063681 Transfer of Acetyl Groups into Mitochondria