EC: 2.3.1.168

dihydrolipoyllysine-residue (2-methylpropanoyl)transferase (2-methylpropanoyl-CoA:enzyme-N6-(dihydrolipoyl)lysine S-(2-methylpropanoyl)transferase)

enzyme lambda metabolic reaction experiment

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:MTBH37RV_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:MOUSE_PWY3DJ-0 isoleucine degradation
BioCyc:MOUSE_PWY3DJ-6 Leucine Catabolism
BioCyc:BSUB_VALDEG-PWY L-valine degradation I
BioCyc:BSUB_PYRUVDEHYD-PWY pyruvate decarboxylation to acetyl CoA
BioCyc:HUMAN_PWY66-428 threonine degradation
BioCyc:HUMAN_VALDEG-PWY valine degradation
BioCyc:MOUSE_PWY-5046 branched-chain α-keto acid dehydrogenase complex
BioCyc:BSUB_ILEUDEG-PWY L-isoleucine degradation I
BioCyc:BSUB_PWY-5130 2-oxobutanoate degradation I
BioCyc:HUMAN_PWY-5130 2-oxobutanoate degradation
BioCyc:META_LEU-DEG2-PWY L-leucine degradation I
BioCyc:MTBH37RV_VALDEG-PWY L-valine degradation I
BioCyc:MOUSE_PWY3DJ-86 valine degradation
BioCyc:BSUB_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-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-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-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-BTA-70895 Branched-chain amino acid catabolism
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-CEL-1430728 Metabolism
Reactome:R-HSA-1430728 Metabolism
WikiPathways:WP4686 Leucine, isoleucine and valine metabolism
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: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: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:SMP0087480 Propanoate Metabolism
PathBank:SMP0087516 Threonine and 2-Oxobutanoate Degradation
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