EC: 1.1.1.178

3-hydroxy-2-methylbutyryl-CoA dehydrogenase ((2S,3S)-3-hydroxy-2-methylbutanoyl-CoA:NAD+ oxidoreductase)

enzyme lambda metabolic reaction experiment
uniprot:O70351 [3-hydroxy-2-methylbutyryl-CoA dehydrogenase(Enzyme) wildtype]

NAD+ + (S)_3_Hydroxybutanoyl_CoA -> NADH + Acetoacetyl_CoA + H+
(( (Vmax * S1) ) / (Km + S1)) buffer: 50 mM Tris-H3PO4, 150 mM KCl, 0.2 mg/ml BSA, 0.7 U/ml Crotonase
PH: 9
Temperature: 25
uniprot:O70351 [3-hydroxy-2-methylbutyryl-CoA dehydrogenase(Enzyme) wildtype]

NAD+ + (2S,3S)_3_Hydroxy_2_methylbutanoyl_CoA -> H+ + NADH + 2_Methylacetoacetyl_CoA
(( (Vmax * S2) ) / (Km + S2)) buffer: 50 mM Tris-H3PO4, 150 mM KCl, 0.2 mg/ml BSA, 0.7 U/ml Crotonase
PH: 9
Temperature: 25
uniprot:O70351 [3-hydroxy-2-methylbutyryl-CoA dehydrogenase(Enzyme) wildtype]

(2S,3S)_3_Hydroxy_2_methylbutanoyl_CoA + NAD+ -> H+ + 2_Methylacetoacetyl_CoA + NADH
(( (Vmax * S1) ) / (Km + S1)) buffer: 50 mM Tris-H3PO4, 150 mM KCl, 0.2 mg/ml BSA, 0.7 U/ml Crotonase
PH: 9
Temperature: 25

Pathways

pathway id name
BioCyc:HUMAN_ILEUDEG-PWY isoleucine degradation
BioCyc:META_FAO-PWY fatty acid β-oxidation I
BioCyc:META_ILEUDEG-PWY L-isoleucine degradation I
BioCyc:MOUSE_PWY3DJ-0 isoleucine degradation
BioCyc:MOUSE_PWY-5109 2-methylbutyrate biosynthesis
BioCyc:HUMAN_FAO-PWY fatty acid β-oxidation
BioCyc:MOUSE_FAO-PWY fatty acid β-oxidation I
BioCyc:MOUSE_PWY-5136 fatty acid β-oxidation II (core pathway)
Reactome:R-BTA-1430728 Metabolism
Reactome:R-BTA-71291 Amino acid and derivative metabolism
Reactome:R-HSA-70895 Branched-chain amino acid catabolism
Reactome:R-HSA-72306 tRNA processing
Reactome:R-HSA-6785470 tRNA processing in the mitochondrion
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-HSA-71291 Amino acid and derivative metabolism
Reactome:R-HSA-8953854 Metabolism of RNA
Reactome:R-HSA-6787450 tRNA modification in the mitochondrion
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-HSA-1430728 Metabolism
Reactome:R-HSA-72312 rRNA processing
Reactome:R-HSA-8868766 rRNA processing in the mitochondrion
WikiPathways:WP4686 Leucine, isoleucine and valine metabolism
PathBank:SMP0120440 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency
PathBank:SMP0120444 3-Methylglutaconic Aciduria Type IV
PathBank:SMP0120516 Maple Syrup Urine Disease
PathBank:SMP0120589 Isovaleric Acidemia
PathBank:SMP0000032 Valine, Leucine, and Isoleucine Degradation
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:SMP0000544 Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD)
PathBank:SMP0000568 Short-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (SCHAD)
PathBank:SMP0063612 Fatty Acid Elongation In Mitochondria
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:SMP0120608 Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase Deficiency (LCHAD)
PathBank:SMP0120632 Short-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency (SCHAD)
PathBank:SMP0000054 Fatty Acid Elongation in Mitochondria
PathBank:SMP0000138 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency
PathBank:SMP0000140 3-Methylglutaconic Aciduria Type III
PathBank:SMP0000238 Isovaleric Aciduria
PathBank:SMP0000523 Isobutyryl-CoA Dehydrogenase Deficiency
PathBank:SMP0063641 Mitochondrial Beta-Oxidation of Short Chain Saturated Fatty Acids
PathBank:SMP0063689 Valine, Leucine, and Isoleucine Degradation
PathBank:SMP0120441 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I
PathBank:SMP0120525 Methylmalonic Aciduria
PathBank:SMP0120537 Propionic Acidemia
PathBank:SMP0120586 3-Hydroxyisobutyric Acid Dehydrogenase Deficiency
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:SMP0120442 3-Methylglutaconic Aciduria Type I
PathBank:SMP0120587 3-Hydroxyisobutyric Aciduria
PathBank:SMP0000236 Propionic Acidemia
PathBank:SMP0000139 3-Methylglutaconic Aciduria Type I
PathBank:SMP0000480 Mitochondrial Beta-Oxidation of Short Chain Saturated Fatty Acids
PathBank:SMP0000522 3-Hydroxyisobutyric Aciduria
PathBank:SMP0000635 Valproic Acid Metabolism Pathway