2.7.11.4 (2R)-2-chloro-3-phenylpropanoic acid - 213705 2.7.11.4 (2S)-2-chloro-3-phenylpropanoic acid - 213704 2.7.11.4 (R)-alpha-chloroisocaproate - 213703 2.7.11.4 (S)-alpha-chloroisocaproate - 213702 2.7.11.4 2-(N-Morpholino)propane sulfonate buffer - 29353 2.7.11.4 2-Chloroisohexanoate no inhibition by (R)(-)-2-chloroisopentanoate; potassium phosphate increases sensitivity to this inhibitor 4199 2.7.11.4 2-Chloroisohexanoate no inhibition by (R)(-)-2-chloroisopentanoate; potassium phosphate increases sensitivity to this inhibitor; (R)(+)-isomer is twice as effective as (S)(-)-isomer; site-specific inhibitor 4199 2.7.11.4 2-Chloroisohexanoate - 4199 2.7.11.4 2-Chloroisohexanoate ATP does not protect; i.e. 2-chloro-4-methylpentanoate, strong 4199 2.7.11.4 2-Chloroisohexanoate enhanced by monovalent cations and further enhanced by phosphate 4199 2.7.11.4 2-Chloroisohexanoate 50% inhibition at 0.014 mM, no inhibition with histone II-S as substrate 4199 2.7.11.4 2-Chloroisohexanoate 50% inhibition at 0.014 mM 4199 2.7.11.4 2-oxo-3-methylpentanoate more effective than 2-oxoisopentanoate 2813 2.7.11.4 2-oxo-3-methylpentanoate - 2813 2.7.11.4 2-oxobutanoate - 390 2.7.11.4 2-oxohexanedioate - 10490 2.7.11.4 2-Oxohexanoate - 1100 2.7.11.4 2-oxoisocaproate more effective than 2-oxo-3-methylpentanoate and 2-oxoisopentanoate 1174 2.7.11.4 2-oxoisocaproate - 1174 2.7.11.4 2-oxoisocaproate kinetics, 40% inhibition at 0.065 mM 1174 2.7.11.4 2-oxoisopentanoate less effective than 2-oxoisohexanoate and 2-oxo-3-methylpentanoate 1270 2.7.11.4 2-oxoisopentanoate - 1270 2.7.11.4 2-oxopentanoate - 968 2.7.11.4 2-oxopentanoate kinetics 968 2.7.11.4 3,6-dichlorobenzo[b]thiophene-2-carboxylic acid - 213698 2.7.11.4 3-chloro-6-fluorobenzo[b]thiophene-2-carboxylic acid - 213699 2.7.11.4 3-methyl-2-oxobutanoate - 829 2.7.11.4 4-(2-Thienyl)-2-oxo-3-butenoate 2 mM 43396 2.7.11.4 4-(3-Thienyl)-2-oxo-3-butenoate 2 mM 15545 2.7.11.4 4-hydroxyphenylacetate - 1708 2.7.11.4 4-hydroxyphenyllactate weak 10530 2.7.11.4 4-hydroxyphenylpyruvate - 423 2.7.11.4 4-hydroxyphenylpyruvate very weak: 3-hydroxyphenylpyruvate 423 2.7.11.4 4-methyl-2-oxopentanoate - 722 2.7.11.4 acetate weak, in vivo and in vitro 47 2.7.11.4 acetoacetyl-CoA - 187 2.7.11.4 acetoacetyl-CoA 40% inhibition at 0.01 mM 187 2.7.11.4 acyl-CoA - 492 2.7.11.4 ADP - 13 2.7.11.4 ADP competitive 13 2.7.11.4 ADP kinetics 13 2.7.11.4 ADP product inhibition 13 2.7.11.4 ADP 50% inhibition at 0.4 mM, inhibition can be reversed by 2 mM Mg2+ 13 2.7.11.4 alpha-Chloroisocaproate inhibits the enzyme by releasing it from the BCKD complex via dissociation 10573 2.7.11.4 alpha-Chloroisocaproate - 10573 2.7.11.4 alpha-Ketoisocaproate inhibits the enzyme by releasing it from the BCKD complex via dissociation 4785 2.7.11.4 alpha-ketoisovalerate inhibits the enzyme by releasing it from the BCKD complex via dissociation 2643 2.7.11.4 ATP - 4 2.7.11.4 ATP 50% inhibition at 0.2 mM, inhibition can be reversed by 2 mM Mg2+ 4 2.7.11.4 branched-chain 2-oxo acids - 20938 2.7.11.4 Ca2+ weak 15 2.7.11.4 Ca2+ - 15 2.7.11.4 CDP 50% inhibition at 0.4 mM, inhibition can be reversed by 2 mM Mg2+ 212 2.7.11.4 Clofibric acid - 8069 2.7.11.4 Clofibric acid in vivo and in vitro 8069 2.7.11.4 CoA - 18 2.7.11.4 CTP 50% inhibition at 0.25 mM, inhibition can be reversed by 2 mM Mg2+ 60 2.7.11.4 dexamethasone decreases enzyme expression level in renal tubule cells 1159 2.7.11.4 Dichloroacetate weak 1160 2.7.11.4 Dichloroacetate - 1160 2.7.11.4 Dichloroacetate ATP slightly protects 1160 2.7.11.4 Dichloroacetate 50% inhibition at 1.8 mM 1160 2.7.11.4 diphosphate - 17 2.7.11.4 Furfurylidenepyruvate 1.85 mM 12533 2.7.11.4 GDP 50% inhibition at 0.2 mM, inhibition can be reversed by 2 mM Mg2+ 53 2.7.11.4 GTP 50% inhibition at 0.06 mM, inhibition can be reversed by 2 mM Mg2+ 37 2.7.11.4 heparin - 227 2.7.11.4 heparin 40% inhibition at 0.012 mg/ml 227 2.7.11.4 heparin 50% inhibition at 0.002 mM 227 2.7.11.4 Hydrocinnamic acid - 12555 2.7.11.4 isobutyryl-CoA - 842 2.7.11.4 isovaleryl-CoA - 784 2.7.11.4 malonyl-CoA - 76 2.7.11.4 methylhydrocinnamic acid - 213706 2.7.11.4 methylmalonyl-CoA - 790 2.7.11.4 methylmalonyl-CoA 40% inhibition at 0.2 mM 790 2.7.11.4 Mg2+ at concentrations above 1.5 mM, activation below 6 2.7.11.4 MgATP2- - 108 2.7.11.4 additional information no inhibition by 2-chloropropionate 2 2.7.11.4 additional information no inhibition by GTP 2 2.7.11.4 additional information no inhibition by acetyl-CoA; no inhibition by coenzyme A 2 2.7.11.4 additional information no inhibition by lactate 2 2.7.11.4 additional information - 2 2.7.11.4 additional information no inhibition by acetate; no inhibition by acetyl-CoA; no inhibition by lactate; no inhibition by methylcrotonyl-CoA, beta-hydroxy-beta-methylglutaryl-CoA, crotonyl-CoA, octanoyl-CoA, succinyl-CoA, propionyl-CoA, 0.1 mM each, propionate, beta-hydroxybutyrate, acetoacetate, malonate, alpha-ketomalonate, succinate, citrate, oxaloacetate, FAD+, NADPH, 2 mM; no inhibition by NADH, NAD+ 1 mM each 2 2.7.11.4 additional information no inhibition by DL-leucine 2 2.7.11.4 additional information binding of thiamin diphosphate cofactor to branched-chain alpha-keto acid decarboxylase/dehydrogenase, which induces a phosphorylation loop conformation change, inhibits the phosphorylation of the protein by the BCKD kinase, no inhibition of phosphorylation of mutant R287A, D295A, Y300F, and R301A E1B components 2 2.7.11.4 additional information clofibric acid and thiamine diphosphate do not affect the protein-protein interaction of BDK with the BCKD complex 2 2.7.11.4 additional information negligible inhibition by butanoic acid and dodecanoic acid 2 2.7.11.4 additional information no inhibition by coenzyme A; no inhibition by isovaleryl-CoA 2 2.7.11.4 additional information no inhibition by isovaleryl-CoA 2 2.7.11.4 N,N'-(1,2,5-oxadiazole-3,4-diyl)bis(3,6-dichlorobenzo[b]thiophene-2-carboxamide - 213701 2.7.11.4 N-(4-acetamido-1,2,5-oxadiazol-3-yl)-3,6-dichlorobenzo[b]thiophene-2-carboxamide - 213700 2.7.11.4 n-Octanoate - 6004 2.7.11.4 n-Octanoate 40% inhibition at 0.5 mM 6004 2.7.11.4 NADP+ - 10 2.7.11.4 NADP+ 40% inhibition at 1.5 mM 10 2.7.11.4 octanoic acid - 1649 2.7.11.4 phenylacetate - 776 2.7.11.4 phenylacetate strong 776 2.7.11.4 phenylbutyrate - 20805 2.7.11.4 Phenyllactate - 4045 2.7.11.4 Phenyllactate strong 4045 2.7.11.4 phenylpyruvate in vivo and in vitro 198 2.7.11.4 phenylpyruvic acid - 8175 2.7.11.4 pyruvate - 31 2.7.11.4 pyruvate weak 31 2.7.11.4 thiamine - 642 2.7.11.4 thiamine diphosphate inhibits phosphorylation of wild-type E1, mutant E1-S303A and mutant E1-D296A/S303A, but not phosphorylation of mutant E1-H292A 91 2.7.11.4 thiamine diphosphate - 91 2.7.11.4 thiamine diphosphate Ca2+-dependent inhibition 91 2.7.11.4 trioctanoin - 11031 2.7.11.4 UDP 50% inhibition at 0.25 mM, inhibition can be reversed by 2 mM Mg2+ 26 2.7.11.4 UTP 50% inhibition at 0.1 mM, inhibition can be reversed by 2 mM Mg2+ 65