| Entry |
|
|---|
| Name |
3-methyl-2-oxobutanoate dehydrogenase
(2-methylpropanoyl-transferring);
2-oxoisocaproate dehydrogenase;
2-oxoisovalerate (lipoate) dehydrogenase;
3-methyl-2-oxobutanoate dehydrogenase (lipoamide);
3-methyl-2-oxobutanoate:lipoamide oxidoreductase (decarboxylating
and acceptor-2-methylpropanoylating);
alpha-keto-alpha-methylvalerate dehydrogenase;
alpha-ketoisocaproate dehydrogenase;
alpha-ketoisocaproic dehydrogenase;
alpha-ketoisocaproic-alpha-keto-alpha-methylvaleric dehydrogenase;
alpha-ketoisovalerate dehydrogenase;
alpha-oxoisocaproate dehydrogenase;
BCKDH;
BCOAD;
branched chain keto acid dehydrogenase;
branched-chain (-2-oxoacid) dehydrogenase (BCD);
branched-chain 2-keto acid dehydrogenase;
branched-chain 2-oxo acid dehydrogenase;
branched-chain alpha-keto acid dehydrogenase;
branched-chain alpha-oxo acid dehydrogenase;
branched-chain keto acid dehydrogenase;
branched-chain ketoacid dehydrogenase;
dehydrogenase, 2-oxoisovalerate (lipoate);
dehydrogenase, branched chain alpha-keto acid |
|---|
| Class |
Oxidoreductases;
Acting on the aldehyde or oxo group of donors;
With a disulfide as acceptor
 |
|---|
| Sysname |
3-methyl-2-oxobutanoate:[dihydrolipoyllysine-residue
(2-methylpropanoyl)transferase]-lipoyllysine 2-oxidoreductase
(decarboxylating, acceptor-2-methylpropanoylating) |
|---|
| Reaction(IUBMB) |
3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue
(2-methylpropanoyl)transferase] lipoyllysine =
[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]
S-(2-methylpropanoyl)dihydrolipoyllysine + CO2 [RN:R01701] |
|---|
| Reaction(KEGG) |
R01701;
(other) R01702 R04225 R07599 R07600 R07601 R07602 R07603 R07604
 |
|---|
| Substrate |
3-methyl-2-oxobutanoate [CPD:C00141];
[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]
lipoyllysine |
|---|
| Product |
[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]
S-(2-methylpropanoyl)dihydrolipoyllysine [CPD:C15977];
CO2 [CPD:C00011] |
|---|
| Cofactor |
Thiamin diphosphate [CPD:C00068] |
|---|
| Comment |
Contains thiamine diphosphate. It acts not only on
3-methyl-2-oxobutanaoate, but also on 4-methyl-2-oxopentanoate and
(S)-3-methyl-2-oxopentanoate, so that it acts on the 2-oxo acids
that derive from the action of transaminases on valine, leucine and
isoleucine. It is a component of the multienzyme
3-methyl-2-oxobutanoate dehydrogenase complex in which multiple
copies of it are bound to a core of molecules of EC 2.3.1.168,
dihydrolipoyllysine-residue (2-methylpropanoyl)transferase, which
also binds multiple copies of EC 1.8.1.4, dihydrolipoyl
dehydrogenase. It does not act on free lipoamide or lipoyllysine,
but only on the lipoyllysine residue in EC 2.3.1.168. |
|---|
| Pathway |
PATH: ec00280 Valine, leucine and isoleucine degradation
PATH: ec01100 Metabolic pathways |
|---|
| Orthology |
KO: K00166 2-oxoisovalerate dehydrogenase E1 component, alpha
subunit
KO: K00167 2-oxoisovalerate dehydrogenase E1 component, beta
subunit
KO: K11381 2-oxoisovalerate dehydrogenase E1 component |
|---|
| Genes |
HSA: 593(BCKDHA) 594(BCKDHB)
PTR: 462845(BCKDHB) 468889(BCKDHA)
MCC: 704978(BCKDHA) 718472
MMU: 100048676 12039(Bckdha) 12040(Bckdhb)
RNO: 25244(Bckdha) 29711(Bckdhb)
CFA: 474978(BCKDHB) 484488(BCKDHA)
BTA: 282149(BCKDHA) 282150(BCKDHB)
SSC: 100142666(BCKDHA) 100142669(BCKDHB)
ECB: 100065300(BCKDHB) 100068678(BCKDHA)
MDO: 100019333 100023799
OAA: 100081828 100088094
GGA: 395375(BCKDHB)
TGU: 100229803
XLA: 100137712
XTR: 100036656
DRE: 554124(bckdha) 569980(bckdhb)
BFO: BRAFLDRAFT_224333
CIN: 100179963 100184456
SPU: 583728 592151
DME: Dmel_CG17691 Dmel_CG8199
DPO: Dpse_GA20891 Dpse_GA29202
DAN: Dana_GF18476
DER: Dere_GG16874 Dere_GG23089
DPE: Dper_GL21161 Dper_GL22326
DSE: Dsec_GM10081 Dsec_GM23814
DSI: Dsim_GD17911 Dsim_GD18623
DYA: Dyak_GE15211 Dyak_GE25960
DGR: Dgri_GH13348 Dgri_GH18279
DMO: Dmoj_GI18255 Dmoj_GI24087
AGA: AgaP_AGAP003136 AgaP_AGAP007531
AAG: AaeL_AAEL008366
CQU: CpipJ_CPIJ011779 CpipJ_CPIJ014532
AME: 409306 412548
NVI: 100116926 100119698 100123532
TCA: 660327 663575
API: 100164806 100165320
ISC: IscW_ISCW018614 IscW_ISCW019115
CEL: F27D4.5(tag-173) Y39E4A.3(Transketolase)
CBR: CBG00293 CBG00294 CBG15770 CBG15799
BMY: Bm1_00280 Bm1_56495
NVE: NEMVE_v1g183894 NEMVE_v1g222088
HMG: 100204367 100209249
TAD: TRIADDRAFT_25751 TRIADDRAFT_63370
ATH: AT1G21400 AT1G55510 AT3G13450(DIN4) AT5G09300 AT5G34780
POP: POPTR_1074695 POPTR_1077225 POPTR_559310
RCU: RCOM_0342870 RCOM_0559760 RCOM_1187670
VVI: 100245451 100248427(GSVIVT00024844001) 100258429
OSA: 4342508(Os07g0170100) 4351684(Os12g0183100)
ZMA: 100191513(pco079304) 100273293(pco118301) 100273630
PPP: PHYPADRAFT_138954 PHYPADRAFT_221150
CRE: CHLREDRAFT_102917
CME: CMH008C CML168C
YLI: YALI0D08690g YALI0F05038g
NCR: NCU03913 NCU09864
PAN: PODANSg2968 PODANSg3246
MGR: MGG_03840 MGG_06511
FGR: FG08622.1 FG09240.1
ANI: AN1726.2 AN8559.2
AFM: AFUA_3G01450 AFUA_6G08830
AOR: AO090001000555 AO090023000349
ANG: An09g02700 An13g03150
AFV: AFLA_107590 AFLA_128600
PCS: Pc12g08790 Pc12g11200
NFI: NFIA_002400 NFIA_054480
CIM: CIMG_01166 CIMG_01357
URE: UREG_01109 UREG_01280
SSL: SS1G_02218 SS1G_09548
BFU: BC1G_04319 BC1G_14088
CNE: CNA03850 CNE02620
CNB: CNBA3660 CNBE2640
LBC: LACBIDRAFT_230442 LACBIDRAFT_319398
UMA: UM05673.1
MBR: MONBRDRAFT_22737 MONBRDRAFT_38974
DDI: DDB_0230185(bkdB) DDB_0230190(bkdA)
PFA: PF13_0070 PFE0225w
PFD: PFDG_00426 PFDG_02699
PFH: PFHG_02354 PFHG_02753
PYO: PY03843 PY05086
PCB: PC000407.01.0 PC000864.02.0
PBE: PB000202.00.0 PB001062.02.0
PKN: PKH_102840 PKH_141180
PVX: PVX_097790 PVX_122460
TAN: TA06600 TA10340
TPV: TP01_0956 TP04_0719
BBO: BBOV_III008200 BBOV_IV008190
TGO: TGME49_039490 TGME49_092100 TGME49_114400
TET: TTHERM_00325580 TTHERM_00704010
PTM: GSPATT00012197001 GSPATT00014995001 GSPATT00015615001
GSPATT00031734001
TBR: Tb10.70.2900 Tb10.70.7550
TCR: 506295.160 506853.50 508781.110 511469.100
LMA: LmjF21.1430 LmjF35.0050
LIF: LinJ21.1100 LinJ35.0150
LBZ: LbrM21_V2.1810 LbrM34_V2.0090
PTI: PHATRDRAFT_11021 PHATRDRAFT_9476
TPS: THAPSDRAFT_32067 THAPSDRAFT_795
PLU: plu1883
PAY: PAU_02673
SML: Smlt3207
SMT: Smal_2640
PAE: PA2247(bkdA1) PA2248(bkdA2)
PAU: PA14_35520(bkdA2) PA14_35530(bkdA1)
PAP: PSPA7_2993(bkdA2) PSPA7_2994(bkdA1)
PAG: PLES_30561(bkdA2) PLES_30571(bkdA1)
PPU: PP_4401(bkdA1) PP_4402(bkdA2)
PPF: Pput_1452 Pput_1453
PPG: PputGB1_3962 PputGB1_3963
PPW: PputW619_3742 PputW619_3743
PFL: PFL_2533 PFL_2534
PFO: Pfl01_3463 Pfl01_3464
PFS: PFLU3964 PFLU3965
PEN: PSEEN3853(bkdA1) PSEEN3854(bkdA2)
SON: SO_2339 SO_2340
SDN: Sden_1786 Sden_1787 Sden_3017
SFR: Sfri_1935 Sfri_1936
SAZ: Sama_1709 Sama_1710 Sama_2795
SBL: Sbal_2221 Sbal_2222
SBM: Shew185_2149 Shew185_2150
SBN: Sbal195_2199 Sbal195_2200
SBP: Sbal223_2234 Sbal223_2235
SLO: Shew_0901 Shew_1925 Shew_1926
SPC: Sputcn32_1885 Sputcn32_1886
SSE: Ssed_2328 Ssed_2329
SPL: Spea_2245 Spea_2246
SHE: Shewmr4_2027 Shewmr4_2028
SHM: Shewmr7_1947 Shewmr7_1948
SHN: Shewana3_2130 Shewana3_2131
SHW: Sputw3181_2122 Sputw3181_2123
SHL: Shal_2233 Shal_2234
SWD: Swoo_1035 Swoo_2278 Swoo_2279
SWP: swp_2605 swp_2606
ILO: IL0177(acoA) IL1679 IL1680
CPS: CPS_1582 CPS_1583
PHA: PSHAa1631(bkdA2) PSHAa1632(BckdhA)
PAT: Patl_2036 Patl_2037
MAQ: Maqu_1381 Maqu_1382
AMC: MADE_01954 MADE_01955
PIN: Ping_2782
LPN: lpg2581
LPF: lpl2503
LPP: lpp2633
LPC: LPC_0566
HCH: HCH_03686 HCH_03687 HCH_06123
MMW: Mmwyl1_3123
KKO: Kkor_1768
BMA: BMAA2012 BMAA2013
BMV: BMASAVP1_1035 BMASAVP1_1036
BML: BMA10229_1321 BMA10229_1322
BMN: BMA10247_A2301 BMA10247_A2302
BPS: BPSS2272(bkdA2) BPSS2273(bkdA1)
BPM: BURPS1710b_A1410(bkdA2) BURPS1710b_A1411(bkdA1)
BPL: BURPS1106A_A3066 BURPS1106A_A3067
BPD: BURPS668_A3192 BURPS668_A3193
BTE: BTH_II2303 BTH_II2304
BUR: Bcep18194_A4360 Bcep18194_A4361
BCN: Bcen_0762 Bcen_0763
BCH: Bcen2424_1243 Bcen2424_1244
BCM: Bcenmc03_1216 Bcenmc03_1217
BCJ: BCAL1212(bkdA1) BCAL1213(bkdA2)
BAM: Bamb_1123 Bamb_1124
BAC: BamMC406_1134 BamMC406_1135
BGL: bglu_2g02640 bglu_2g02650
BPT: Bpet3510(bkdA2) Bpet3511(bkdA1)
RFR: Rfer_3553 Rfer_3554
POL: Bpro_0273 Bpro_0274
VAP: Vapar_1010 Vapar_1011
BBA: Bd0972 Bd0974
ADE: Adeh_1826 Adeh_1827
ACP: A2cp1_2103 A2cp1_2104
AFW: Anae109_1993 Anae109_1994
ANK: AnaeK_2033 AnaeK_2034
MXA: MXAN_4564 MXAN_4565
SCL: sce1570(pdhB1) sce1571(pdhA) sce8001
HOH: Hoch_2652 Hoch_3236 Hoch_3237
MLO: mll4472 mll4473
SME: SMc03201(bkdAa) SMc03202(bkdAb)
SMD: Smed_2826 Smed_2827 Smed_4110
ATU: Atu3472(bkdA2) Atu3473(bkdA1)
ATC: AGR_L_2716 AGR_L_2718
RET: RHE_PC00070(bkDa1) RHE_PC00071(bkDa2)
RLE: pRL100303 pRL100304 pRL80083
RLT: Rleg2_4385 Rleg2_4386
RLG: Rleg_6502 Rleg_6503
RHI: NGR_b11450 NGR_c29890(bkdA1) NGR_c29900(bkdA2)
BME: BMEII0061 BMEII0216 BMEII0747 BMEII0748
BMI: BMEA_B0497 BMEA_B0498 BMEA_B1091
BMF: BAB2_0031 BAB2_0714 BAB2_0715
BMB: BruAb2_0032 BruAb2_0699 BruAb2_0700 BruAb2_1025
BMC: BAbS19_II00300 BAbS19_II06660 BAbS19_II06670 BAbS19_II09710
BMS: BRA0032 BRA0524 BRA0525
BMT: BSUIS_B0034 BSUIS_B0519 BSUIS_B0520 BSUIS_B1081
BOV: BOV_A0029 BOV_A0455 BOV_A0456
BCS: BCAN_B0034 BCAN_B0522 BCAN_B0523 BCAN_B1108
BMR: BMI_II1086 BMI_II32 BMI_II518 BMI_II519
OAN: Oant_3551 Oant_3552
BJA: blr6331(bkdA1) blr6332(bkdA2)
XAU: Xaut_1571
CAK: Caul_0582 Caul_0587 Caul_1870 Caul_1871
SIL: SPO0585
SIT: TM1040_2777 TM1040_2778
RSP: RSP_1294
RSH: Rsph17029_2954
RSQ: Rsph17025_2727
RSK: RSKD131_2694
JAN: Jann_3081
RDE: RD1_2926(pdhB)
PDE: Pden_1604 Pden_4757 Pden_4758
DSH: Dshi_2566
MMR: Mmar10_0704 Mmar10_0705
HBA: Hbal_1614 Hbal_1615
NAR: Saro_1045 Saro_1975 Saro_1976
SAL: Sala_1330 Sala_1331 Sala_2962
SWI: Swit_0762 Swit_0781 Swit_0782 Swit_2144 Swit_2145 Swit_3884
ELI: ELI_05155 ELI_05160
BSU: BSU24040(bkdAB) BSU24050(bkdAA)
BHA: BH2762(bfmBAB) BH2763(bfmBAA)
BAN: BA4383(bfmbAb) BA4384(bfmbAa)
BAR: GBAA4383(bfmbAb) GBAA4384(bfmbAa)
BAA: BA_4838 BA_4839
BAT: BAS4066 BAS4067
BAH: BAMEG_4420(bfmbAb) BAMEG_4421(bfmbAa)
BAI: BAA_4402(bfmbAb) BAA_4403(bfmbAa)
BCE: BC4158 BC4159
BCA: BCE_4233(bfmbAb) BCE_4234(bfmbAa)
BCZ: BCZK3913(bfmbAb) BCZK3914(bfmbAa)
BCR: BCAH187_A4291(bfmbAb) BCAH187_A4292(bfmbAa)
BCB: BCB4264_A4271(bfmbAb) BCB4264_A4272(bfmbAa)
BCU: BCAH820_4181(bfmbAb) BCAH820_4182(bfmbAa)
BCG: BCG9842_B0962(bfmbAa) BCG9842_B0963(bfmbAb)
BCQ: BCQ_3948(bfmbAb) BCQ_3949(bfmbAa)
BCX: BCA_4270(bfmbAb) BCA_4271(bfmbAa)
BCY: Bcer98_2855 Bcer98_2856
BTK: BT9727_3904(bfmbAb) BT9727_3905(bfmbAa)
BTL: BALH_3771(bfmbAb) BALH_3772(bfmbAa)
BWE: BcerKBAB4_4002 BcerKBAB4_4003
BLI: BL01504(bkdAA) BL01505(bkdAB)
BLD: BLi02581(bkdAB) BLi02582(bkdAA)
BCL: ABC2450(bkdA2) ABC2451(bkdA1)
BAY: RBAM_022320(bkdAB) RBAM_022330(bkdAA)
BPU: BPUM_2143(bfmBAB) BPUM_2144(bfmBAA)
OIH: OB1865 OB1866
GKA: GK2377 GK2378
GTN: GTNG_2306 GTNG_2307
GWC: GWCH70_2304 GWCH70_2305
GYM: GYMC10_2125 GYMC10_2126
GYC: GYMC61_0265 GYMC61_0266
AFL: Aflv_0962(bkdAA) Aflv_0963(bkdAB)
SAU: SA1347(bfmBAB) SA1348(bfmBAA)
SAV: SAV1516(bfmBAB) SAV1517(bfmBAA)
SAW: SAHV_1504(bfmBAB) SAHV_1505(bfmBAA)
SAM: MW1469(bfmBAB) MW1470(bfmBAA)
SAR: SAR1594(bfmBAB) SAR1595(bfmBAA)
SAS: SAS1455 SAS1456
SAC: SACOL1561 SACOL1562
SAB: SAB1389c SAB1390c
SAA: SAUSA300_1465 SAUSA300_1466
SAX: USA300HOU_1518 USA300HOU_1519(odpA)
SAO: SAOUHSC_01612 SAOUHSC_01613
SAJ: SaurJH9_1575 SaurJH9_1576
SAH: SaurJH1_1608 SaurJH1_1609
SAE: NWMN_1422 NWMN_1423
SAD: SAAV_1509 SAAV_1510
SEP: SE1197 SE1198
SER: SERP1077 SERP1078
SHA: SH1398(bfmBAA) SH1399(bfmBAB)
SSP: SSP1238 SSP1239
SCA: Sca_1138(bfmBAB) Sca_1139(bfmBAA)
LMO: lmo1372 lmo1373
LMF: LMOf2365_1389 LMOf2365_1390
LMH: LMHCC_1198 LMHCC_1199
LMC: Lm4b_01381 Lm4b_01382
LIN: lin1409 lin1410
LWE: lwe1387 lwe1388
LSP: Bsph_3493 Bsph_3494
ESI: Exig_0919 Exig_0920
EAT: EAT1b_0506 EAT1b_0507
BBE: BBR47_23690(bkdA1) BBR47_23700(bkdA2)
PJD: Pjdr2_2321 Pjdr2_2322
LCA: LSEI_1444 LSEI_1445
LCB: LCABL_16670(bkdB) LCABL_16680(bkdA)
EFA: EF1659(bkdB) EF1660(bkdA)
STH: STH2161 STH2162 STH583
ATE: Athe_0704
CSC: Csac_0874
MSM: MSMEG_2467
MMC: Mmcs_1976
MKM: Mkms_2022
MJL: Mjls_1956
NFA: nfa35780
RHA: RHA1_ro03301
RER: RER_04420(acoA)
ROP: ROP_28430
NCA: Noca_4454
SRO: Sros_6420
NML: Namu_2114 Namu_3319
SEN: SACE_3884
STP: Strop_3370
SAQ: Sare_3610
RXY: Rxyl_2479 Rxyl_2480
CTR: CT340(pdhA)
CTA: CTA_0369(pdhA_2)
CTB: CTL0594
CTL: CTLon_0592
CTJ: JALI_3381
CMU: TC0618
CPN: CPn0033(pdhA)
CPA: CP0743
CPJ: CPj0033(pdhA_pdhB)
CPT: CpB0037
CCA: CCA00324(pdhA)
CAB: CAB319
CFE: CF0679(pdhAB)
ABA: Acid345_1786
ACA: ACP_3156
SUS: Acid_0353 Acid_2937 Acid_3687
BTH: BT_0312
BFR: BF1622
BFS: BF1636
PDI: BDI_0687
SRU: SRU_0576 SRU_1966
RMR: Rmar_0991 Rmar_1486 Rmar_1549
CHU: CHU_3183(dxs)
CPI: Cpin_0808 Cpin_1538
PHE: Phep_3824
GFO: GFO_1616
FJO: Fjoh_2840
FPS: FP1028(bfmBA)
FBA: FIC_01383
RBI: RB2501_14694
OTE: Oter_4204
GAU: GAU_1056 GAU_2797
GVI: gll1094
CTS: Ctha_0429 Ctha_2297
RRS: RoseRS_2415 RoseRS_2416
RCA: Rcas_3062 Rcas_3063
CAU: Caur_1692 Caur_1693
CAG: Cagg_2095 Cagg_2096
CHL: Chy400_1833 Chy400_1834
HAU: Haur_2591 Haur_2592
DRA: DR_0029 DR_0030
DGE: Dgeo_2338 Dgeo_2339
DDR: Deide_23440 Deide_23450
TTH: TTC1756 TTC1757
TTJ: TTHA0229 TTHA0230
DTH: DICTH_0301
DTU: Dtur_0419
TTR: Tter_0093 Tter_0094
HMA: rrnAC0149(pdhA1) rrnAC2957(pdhA3)
 |
|---|
| Structures |
PDB: 1DTW 1OLS 1OLU 1OLX 1U5B 1UM9 1UMB 1UMC 1UMD 1V11
1V16 1V1M 1V1R 1WCI 1X7W 1X7X 1X7Y 1X7Z 1X80 2BEU
2BEV 2BEW 2BFB 2BFC 2BFD 2BFE 2BFF 2BP7 2J9F |
|---|
Reference
Authors
Title
Journal
Organism
|
1 [PMID:5656388]
Bowden JA, Connelly JL.
Branched chain alpha-keto acid metabolism. II. Evidence for the
common identity of alpha-ketoisocaproic acid and
alpha-keto-beta-methyl-valeric acid dehydrogenases.
J. Biol. Chem. 243 (1968) 3526-31.
Bos taurus [GN:bta] |
|---|
Reference
Authors
Title
Journal
Organism
|
2 [PMID:5689906]
Connelly JL, Danner DJ, Bowden JA.
Branched chain alpha-keto acid metabolism. I. Isolation,
purification, and partial characterization of bovine liver
alpha-ketoisocaproic:alpha-keto-beta-methylvaleric acid
dehydrogenase.
J. Biol. Chem. 243 (1968) 1198-203.
Bos taurus [GN:bta] |
|---|
Reference
Authors
Title
Journal
Organism
|
3 [PMID:447664]
Danner DJ, Lemmon SK, Besharse JC, Elsas LJ 2nd.
Purification and characterization of branched chain alpha-ketoacid
dehydrogenase from bovine liver mitochondria.
J. Biol. Chem. 254 (1979) 5522-6.
Bos taurus [GN:bta] |
|---|
Reference
Authors
Title
Journal
Organism
|
4 [PMID:283398]
Pettit FH, Yeaman SJ, Reed LJ.
Purification and characterization of branched chain alpha-keto acid
dehydrogenase complex of bovine kidney.
Proc. Natl. Acad. Sci. U. S. A. 75 (1978) 4881-5.
Bos taurus [GN:bta] |
|---|
Reference
Authors
Title
Journal
|
5 [PMID:10966480]
Perham RN.
Swinging arms and swinging domains in multifunctional enzymes:
catalytic machines for multistep reactions.
Annu. Rev. Biochem. 69 (2000) 961-1004. |
|---|
| Other DBs |
ExplorEnz - The Enzyme Database: 1.2.4.4
IUBMB Enzyme Nomenclature: 1.2.4.4
ExPASy - ENZYME nomenclature database: 1.2.4.4
BRENDA, the Enzyme Database: 1.2.4.4
CAS: 9082-72-8 |
|---|
