KEGG   ENZYME: 1.3.8.5
Entry
EC 1.3.8.5                  Enzyme                                 

Name
short-chain 2-methylacyl-CoA dehydrogenase;
ACADSB (gene name);
2-methylacyl-CoA dehydrogenase;
branched-chain acyl-CoA dehydrogenase (ambiguous);
2-methyl branched chain acyl-CoA dehydrogenase;
2-methylbutanoyl-CoA:(acceptor) oxidoreductase;
2-methyl-branched-chain-acyl-CoA:electron-transfer flavoprotein 2-oxidoreductase;
2-methyl-branched-chain-enoyl-CoA reductase
Class
Oxidoreductases;
Acting on the CH-CH group of donors;
With a flavin as acceptor
Sysname
short-chain 2-methylacyl-CoA:electron-transfer flavoprotein 2-oxidoreductase
Reaction(IUBMB)
2-methylbutanoyl-CoA + electron-transfer flavoprotein = (E)-2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H+ [RN:R03169]
Reaction(KEGG)
R03169;
(other) R02661 R03172 R04751
Substrate
2-methylbutanoyl-CoA [CPD:C01033];
electron-transfer flavoprotein [CPD:C04253]
Product
(E)-2-methylbut-2-enoyl-CoA [CPD:C03345];
reduced electron-transfer flavoprotein [CPD:C04570];
H+ [CPD:C00080]
Comment
A flavoprotein (FAD). The mammalian enzyme catalyses an oxidative reaction as a step in the mitochondrial beta-oxidation of short-chain 2-methyl fatty acids and participates in isoleucine degradation. The enzyme from the parasitic helminth Ascaris suum catalyses a reductive reaction as part of a fermentation pathway, shuttling reducing power from the electron-transport chain to 2-methyl branched-chain enoyl CoA.
History
EC 1.3.8.5 created 1992 as EC 1.3.1.52, transferred 2012 to EC 1.3.8.5 (EC 1.3.99.12, created 1986, incorporated 2020), modified 2020
Pathway
ec00071  Fatty acid degradation
ec00280  Valine, leucine and isoleucine degradation
ec01100  Metabolic pathways
ec01110  Biosynthesis of secondary metabolites
Orthology
K09478  short-chain 2-methylacyl-CoA dehydrogenase
K11410  short-chain 2-methylacyl-CoA dehydrogenase
Genes
HSA: 36(ACADSB)
PTR: 745464(ACADSB)
PPS: 100991276(ACADSB)
GGO: 101132470(ACADSB)
PON: 100171571(ACADSB)
NLE: 100603582(ACADSB)
MCC: 707487(ACADSB)
MCF: 102133754(ACADSB)
CSAB: 103216651(ACADSB)
CATY: 105578003(ACADSB)
PANU: 101010148(ACADSB)
RRO: 104658093(ACADSB)
RBB: 108519576(ACADSB)
TFN: 117065359(ACADSB)
PTEH: 111550816(ACADSB)
CJC: 100396089(ACADSB)
SBQ: 101028121(ACADSB)
MMUR: 105880343(ACADSB)
MMU: 66885(Acadsb)
MCAL: 110299023(Acadsb)
MPAH: 110326470(Acadsb)
RNO: 25618(Acadsb)
MCOC: 116103099(Acadsb)
MUN: 110564692
CGE: 100766828(Acadsb)
PLEU: 114692584(Acadsb)
NGI: 103729261(Acadsb)
HGL: 101698912(Acadsb)
CCAN: 109691642(Acadsb)
OCU: 100351868(ACADSB)
TUP: 102474463(ACADSB)
CFA: 477856(ACADSB)
VVP: 112928830(ACADSB)
VLG: 121484200(ACADSB)
AML: 100470228(ACADSB)
UMR: 103657958(ACADSB)
UAH: 113252021(ACADSB)
ORO: 101373794(ACADSB)
ELK: 111145042
MPUF: 101674215(ACADSB)
EJU: 114221858(ACADSB)
MLX: 118027358(ACADSB)
FCA: 101092794(ACADSB)
PTG: 102951023(ACADSB)
PPAD: 109252314(ACADSB)
AJU: 106969392(ACADSB)
HHV: 120235521(ACADSB)
BTA: 504301(ACADSB)
BOM: 102266366(ACADSB)
BIU: 109579124(ACADSB)
BBUB: 102397738(ACADSB)
CHX: 102177588(ACADSB)
OAS: 101102606(ACADSB)
ODA: 120873887(ACADSB)
SSC: 100154810(ACADSB)
CFR: 102517479(ACADSB)
CBAI: 105067638(ACADSB)
CDK: 105085769(ACADSB)
BACU: 102999342(ACADSB)
LVE: 103077062(ACADSB)
OOR: 101289781(ACADSB)
DLE: 111172226(ACADSB)
PCAD: 102992141(ACADSB)
ECB: 100057998(ACADSB)
EAI: 106847757(ACADSB)
MYB: 102253452(ACADSB)
MYD: 102769159(ACADSB)
MMYO: 118669618(ACADSB)
MNA: 107532676(ACADSB)
HAI: 109396916(ACADSB)
DRO: 112300262(ACADSB)
SHON: 118990529(ACADSB)
AJM: 119059542(ACADSB)
MMF: 118632767(ACADSB)
PALE: 102891849(ACADSB)
MJV: 108390573(ACADSB)
TOD: 119234148(ACADSB)
LAV: 100658432(ACADSB)
TMU: 101353141
MDO: 100025277(ACADSB)
SHR: 100925751(ACADSB)
PCW: 110192795(ACADSB)
OAA: 100086999(ACADSB)
GGA: 423947(ACADSB)
PCOC: 116231551(ACADSB)
MGP: 100542282(ACADSB)
CJO: 107316256(ACADSB)
NMEL: 110399180(ACADSB)
APLA: 101793844(ACADSB)
ACYG: 106038988(ACADSB)
TGU: 100217590(ACADSB)
LSR: 110468433(ACADSB)
SCAN: 103813777(ACADSB)
PMOA: 120500006(ACADSB)
GFR: 102040450(ACADSB)
FAB: 101819094(ACADSB)
PHI: 102100927(ACADSB)
PMAJ: 107206820(ACADSB)
CCAE: 111931386(ACADSB)
CCW: 104694570(ACADSB)
ETL: 114070836(ACADSB)
FPG: 101917969(ACADSB)
FCH: 102057808(ACADSB)
CLV: 102096980(ACADSB)
EGZ: 104128327(ACADSB)
NNI: 104022112(ACADSB)
ACUN: 113481576(ACADSB)
PADL: 103918535(ACADSB)
AAM: 106494200(ACADSB)
ASN: 102386096(ACADSB)
AMJ: 102577069(ACADSB)
CPOO: 109324120(ACADSB)
GGN: 109287690(ACADSB)
PSS: 102458654(ACADSB)
CMY: 102942130(ACADSB)
CPIC: 101938408(ACADSB)
TST: 117881058(ACADSB)
CABI: 116834432(ACADSB)
ACS: 100556766(acadsb)
PVT: 110087224(ACADSB)
PBI: 103066261(ACADSB)
PMUR: 107285940(ACADSB)
TSR: 106554066(ACADSB)
PGUT: 117676700(ACADSB)
PMUA: 114597407(ACADSB)
ZVI: 118096516(ACADSB)
GJA: 107116296(ACADSB)
XLA: 494679(acadsb.L)
XTR: 448259(acadsb)
NPR: 108784780(ACADSB)
DRE: 100037342(acadsb) 110437777(acadsb)
SRX: 107746745
SANH: 107684586
IPU: 108273611(acadsb)
PHYP: 113527247(acadsb)
AMEX: 103042655(acadsb)
EEE: 113588401(acadsb)
TRU: 101075946(acadsb)
LCO: 104926628(acadsb)
NCC: 104954036(acadsb)
CGOB: 115024463(acadsb)
ELY: 117246729(acadsb)
PLEP: 121959173(acadsb)
SLUC: 116061434(acadsb)
ECRA: 117937079(acadsb)
PFLV: 114548099(acadsb)
GAT: 120819195(acadsb)
MSAM: 119915473(acadsb)
CUD: 121528185(acadsb)
MZE: 101471898(acadsb)
ONL: 100692418(acadsb)
OAU: 116328816(acadsb)
OLA: 101163855(acadsb)
OML: 112154111(acadsb)
XMA: 102220838(acadsb)
XCO: 114152310(acadsb)
XHE: 116727535(acadsb)
PRET: 103481581(acadsb)
CVG: 107090227(acadsb)
CTUL: 119775960(acadsb)
NFU: 107387801(acadsb)
KMR: 108246997(acadsb)
ALIM: 106516587(acadsb)
AOCE: 111582229(acadsb)
CSEM: 103381955(acadsb)
POV: 109626616(acadsb)
LCF: 108899015(acadsb)
SDU: 111220345(acadsb)
SLAL: 111660219(acadsb)
XGL: 120794321(acadsb)
HCQ: 109523983(acadsb)
BPEC: 110165473(acadsb)
MALB: 109962961(acadsb)
SALP: 112075854
ELS: 105009552(acadsb)
SFM: 108939566(acadsb)
PKI: 111853681(acadsb)
AANG: 118217576(acadsb)
LOC: 102695849(acadsb)
ARUT: 117418434(acadsb)
LCM: 102365761(ACADSB)
CMK: 103185437(acadsb)
RTP: 109927465(acadsb)
BFO: 118414863
BBEL: 109471473
SPU: 593139
APLC: 110988182
SKO: 100378591
DME: Dmel_CG3902(CG3902)
DER: 6545091
DSE: 6618359
DSI: Dsimw501_GD12323(Dsim_GD12323)
DAN: 6493668
DSR: 110185241
DPE: 6595810
DMN: 108153425
DWI: 6645938
DGR: 6557827
DNV: 108650232
DHE: 111592256
DVI: 6624036
CCAT: 101448771
MDE: 101890297
LCQ: 111690724
ACOZ: 120957175
AARA: 120902866
AAG: 5569333
CPII: 120428341
AME: 409712
ACER: 108000837
BIM: 100749552
BBIF: 117208917
BTER: 100649947
CCAL: 108628770
OBB: 114880543
MGEN: 117218232
NMEA: 116431539
CGIG: 122394773
AEC: 105143927
PBAR: 105428815
CFO: 105250026
FEX: 115239064
LHU: 105674413
PGC: 109859061
PCF: 106790286
CSOL: 105365671
MDL: 103579394
CCIN: 107266330
DPA: 109540223
ATD: 109595381
AGB: 108916585
LDC: 111506372
APLN: 108739121
BMOR: 733139(Acade)
BANY: 112051716
PMAC: 106716140
PPOT: 106104020
PXU: 106116729
PRAP: 111002788
ZCE: 119833809
HAW: 110372345
TNL: 113505313
PXY: 105383530
API: 100161123
DNX: 107163553
AGS: 114124068
RMD: 113551881
BTAB: 109039976
DCI: 103511898
CLEC: 106664106
HHAL: 106682813
ZNE: 110839588
CSEC: 111874809
FCD: 110852222
DMK: 116927994
PVM: 113821404
HAME: 121864223
DPTE: 113793263
CSCU: 111633258
CEL: CELE_C55B7.4(acdh-1) CELE_K06A5.6(acdh-3) CELE_T10E9.9(acdh-4)
TSP: Tsp_04649
PCAN: 112563554
BGT: 106074053
GAE: 121384123
CRG: 105329007
MYI: 110442658
OBI: 106881672
LAK: 106173907
NVE: 5508334
EPA: 110246509
ATEN: 116293762
AMIL: 114958115
PDAM: 113681854
SPIS: 111331973
DGT: 114515827
HMG: 100205931
AQU: 100640316
QSU: 112003075
SLB: AWJ20_448
NCR: NCU06543
NTE: NEUTE1DRAFT145014(NEUTE1DRAFT_145014)
SSCK: SPSK_09149
CMT: CCM_00006
MBE: MBM_00834
TASA: A1Q1_02301
ABP: AGABI1DRAFT116215(AGABI1DRAFT_116215)
ABV: AGABI2DRAFT191649(AGABI2DRAFT_191649)
MRT: MRET_3963
DDI: DDB_G0282967(acadsb)
DFA: DFA_08597(acadsb)
FCY: FRACYDRAFT_187779(ACD2)
TPS: THAPSDRAFT_269127(ACD3)
SPAR: SPRG_03679
PALL: UYA_22885
GPB: HDN1F_19750(acdH)
ODI: ODI_R3782
DMA: DMR_42700
DML: Dmul_28490(acdA10)
DAL: Dalk_4346
DAT: HRM2_10010(acd3)
DTO: TOL2_C06730(AcdA)
DOV: DSCO28_50220(acdA)
DWD: DSCW_44620(acdA)
DALK: DSCA_44210(acdA_2)
DLI: dnl_30090(acdA2)
DMM: dnm_037100(acdA3)
DBR: Deba_1947
MNO: Mnod_8045
PGV: SL003B_1080(acdH)
MTU: Rv2500c(fadE19)
MTC: MT2575
MRA: MRA_2526(fadE19)
MTUR: CFBS_2649(fadE19)
MTO: MTCTRI2_2546(fadE19)
MTD: UDA_2500c(fadE19)
MTN: ERDMAN_2750(fadE19)
MTUC: J113_17390
MTUE: J114_13370
MTUH: I917_17645
MTUL: TBHG_02437
MTUT: HKBT1_2640(fadE19)
MTUU: HKBT2_2643(fadE19)
MTQ: HKBS1_2647(fadE19)
MBO: BQ2027_MB2528C(fadE19)
MBT: JTY_2514(fadE19)
MBM: BCGMEX_2512c(fadE19)
MBX: BCGT_2338
MAF: MAF_25150(fadE19)
MMIC: RN08_2771
MCE: MCAN_25391(fadE19)
MCQ: BN44_50491(fadE)
MCV: BN43_40166(fadE)
MCX: BN42_40456(fadE)
MCZ: BN45_50884(fadE)
MPA: MAP_2312c(fadE19)
MAO: MAP4_1511
MAVI: RC58_07510
MAVU: RE97_07510
MIT: OCO_17410
MIA: OCU_17600
MID: MIP_02453
MYO: OEM_15430
MIR: OCQ_15050
MLP: MLM_2830
MMAN: MMAN_24270(fadE19)
MMC: Mmcs_3629
MKM: Mkms_3702
MJL: Mjls_3634
MMI: MMAR_3847(fadE19)
MMAE: MMARE11_37330(fadE19)
MMM: W7S_07370
MLI: MULP_04093(fadE19)
MHAD: B586_08605
MSHG: MSG_03512(fadE19)
MFJ: MFLOJ_51830(fadE19)
MSTO: MSTO_56040(fadE19)
MSIM: MSIM_41300(fadE19)
MSAK: MSAS_17830(fadE19)
MNM: MNVM_04810(fadE19)
MCOO: MCOO_15620
MBAI: MB901379_03415(mmgC_14)
MSEO: MSEO_23110(fadE19)
MPSE: MPSD_39080(fadE19)
MSHO: MSHO_30710(fadE19)
MLJ: MLAC_10850(fadE19)
MBRD: MBRA_33150
MSHJ: MSHI_22950(fadE19)
MVA: Mvan_4089
MGI: Mflv_2554
MPHL: MPHLCCUG_03665(acdA_10)
MVQ: MYVA_3888
MHAS: MHAS_01111(acdA_3)
MMAG: MMAD_38410
MMOR: MMOR_24700
MAIC: MAIC_37140
MALV: MALV_53970
MARZ: MARA_58870
MGAD: MGAD_57880
MHEV: MHEL_04080
MSAR: MSAR_47610
MANY: MANY_21480
MAUB: MAUB_48440
MPOF: MPOR_42230
MPHU: MPHO_21250
MBOK: MBOE_32570
MAB: MAB_4538c
MABB: MASS_4565
MCHE: BB28_23155
MSTE: MSTE_04705
MSAL: DSM43276_04379(mmgC_10)
MJD: JDM601_1405(fadE19)
MTER: 4434518_01298(fadE19)
MMIN: MMIN_29990(fadE19)
MHIB: MHIB_08610(fadE19)
ASD: AS9A_4351
CRD: CRES_1678(fadE8)
CVA: CVAR_2090
CTER: A606_08410
COA: DR71_1563
CPRE: Csp1_08440(mmgC)
CUO: CUROG_07785(mmgC2)
NFA: NFA_50390(fadE43)
RER: RER_18320(fadE)
REY: O5Y_08795
ROP: ROP_61540(fadE)
REQ: REQ_36020
RHB: NY08_3989
RFA: A3L23_02158(acdA_6)
RHS: A3Q41_01220(acdA_3)
RHU: A3Q40_00066(acdA_1)
GBR: Gbro_1359
GOR: KTR9_1273
GRU: GCWB2_06965(mmgC7)
GOM: D7316_01863(acdA_1)
TPR: Tpau_3319
SCO: SCO2779(acdH)
SALB: XNR_4209
SMA: SAVERM_5275(fadE4)
SGR: SGR_4756
SGB: WQO_11760
SCB: SCAB_58001(acdH)
SHY: SHJG_4280
SMAL: SMALA_2810
SCT: SCAT_1857(yngJ)
SFA: Sfla_4110
SBH: SBI_06919
SVE: SVEN_2568
SDV: BN159_5499(yngJ1)
SALS: SLNWT_2338
STRP: F750_2606
SFI: SFUL_2370
SALU: DC74_3256
SALL: SAZ_17345
STRE: GZL_05657
SLD: T261_5312
STRM: M444_13330
SAMB: SAM23877_2814(yngJ)
SPRI: SPRI_4763
SRW: TUE45_03394(mmgC_7)
SLE: sle_44610(sle_44610)
SRN: A4G23_01831(mmgC_3)
STRD: NI25_24960
SLAU: SLA_2529
SALF: SMD44_03078(acdH)
SALJ: SMD11_4398(acdH)
SLX: SLAV_23940(mmgC10)
SFK: KY5_2919
SGE: DWG14_05329(mmgC_4)
SRJ: SRO_4690
SNF: JYK04_03391(mmgC_3)
SHUN: DWB77_04913(mmgC_5)
SCYG: S1361_15210(mmgC6)
KSK: KSE_26850(fadE3)
MTS: MTES_3611(fadE19)
MOO: BWL13_01138(mmgC_3)
MLV: CVS47_01278(mmgC_2)
MOY: CVS54_01933(acdA)
ART: Arth_1387
ARM: ART_4230
ACH: Achl_1405
KRH: KRH_22750(fadE)
BCV: Bcav_3850
LMOI: VV02_10345
XCE: Xcel_2102
IDO: I598_1783(acdA)
CFI: Celf_2557
SERJ: SGUI_3118
BLIN: BLSMQ_0975
MPH: MLP_10090(fadE)
NCA: Noca_3506
NDK: I601_0190(acdA_1)
PSIM: KR76_20105
KFL: Kfla_1418
TFU: Tfu_0946
NDA: Ndas_3266
NAL: B005_0221
STRR: EKD16_09530(mmgC4)
TCU: Tcur_1974
SRO: Sros_6410
NCX: Nocox_29015(acdA6)
TBI: Tbis_2151
FAL: FRAAL3160
ACE: Acel_0405
NML: Namu_1730
GOB: Gobs_1474
SEN: SACE_7037(acdH)
SACC: EYD13_16915(mmgC6)
AMD: AMED_7920
AOI: AORI_6737(bcd)
AMQ: AMETH_1831(acdH) AMETH_5735(acdH)
AMYY: YIM_05860(mmgC3)
AORI: SD37_06945
PSEA: WY02_14630
PSEE: FRP1_27840
PSEH: XF36_26125
PAUT: Pdca_64080
AMI: Amir_6228
SESP: BN6_74940
KAL: KALB_7771
ACTI: UA75_26745
ACAD: UA74_26160
AHG: AHOG_24035(mmgC2)
ALO: CRK59629
SAQ: Sare_0034
MIL: ML5_0061
ASE: ACPL_149(acdH)
AMS: AMIS_300
ACTN: L083_0037(fadE)
AFS: AFR_00195
ACTS: ACWT_0034
CAI: Caci_2187
SNA: Snas_6416
RXY: Rxyl_1707
CWO: Cwoe_0232
AFO: Afer_0732
SUS: Acid_5310
BLQ: L21SP5_00851(mmgC_1)
CABY: Cabys_2853
 » show all
Reference
1  [PMID:6401712]
  Authors
Ikeda Y, Dabrowski C, Tanaka K.
  Title
Separation and properties of five distinct acyl-CoA dehydrogenases from rat liver mitochondria. Identification of a new 2-methyl branched chain acyl-CoA dehydrogenase.
  Journal
J Biol Chem 258:1066-76 (1983)
Reference
2  [PMID:3988734]
  Authors
Komuniecki R, Fekete S, Thissen-Parra J.
  Title
Purification and characterization of the 2-methyl branched-chain Acyl-CoA dehydrogenase, an enzyme involved in NADH-dependent enoyl-CoA reduction in anaerobic mitochondria of the nematode, Ascaris suum.
  Journal
J Biol Chem 260:4770-7 (1985)
Reference
3  [PMID:2736251]
  Authors
Komuniecki R, McCrury J, Thissen J, Rubin N.
  Title
Electron-transfer flavoprotein from anaerobic Ascaris suum mitochondria and its role in NADH-dependent 2-methyl branched-chain enoyl-CoA reduction.
  Journal
Biochim Biophys Acta 975:127-31 (1989)
DOI:10.1016/S0005-2728(89)80210-5
Reference
4  [PMID:10989435]
  Authors
Vockley J, Mohsen al-W A, Binzak B, Willard J, Fauq A.
  Title
Mammalian branched-chain acyl-CoA dehydrogenases: molecular cloning and characterization of recombinant enzymes.
  Journal
Methods Enzymol 324:241-58 (2000)
DOI:10.1016/s0076-6879(00)24236-5
Reference
5  [PMID:11013134]
  Authors
Andresen BS, Christensen E, Corydon TJ, Bross P, Pilgaard B, Wanders RJ, Ruiter JP, Simonsen H, Winter V, Knudsen I, Schroeder LD, Gregersen N, Skovby F
  Title
Isolated 2-methylbutyrylglycinuria caused by short/branched-chain acyl-CoA dehydrogenase deficiency: identification of a new enzyme defect, resolution of its molecular basis, and evidence for distinct acyl-CoA dehydrogenases in isoleucine and valine metabolism.
  Journal
Am J Hum Genet 67:1095-103 (2000)
DOI:10.1086/303105
Other DBs
ExplorEnz - The Enzyme Database: 1.3.8.5
IUBMB Enzyme Nomenclature: 1.3.8.5
ExPASy - ENZYME nomenclature database: 1.3.8.5
BRENDA, the Enzyme Database: 1.3.8.5
LinkDB

DBGET integrated database retrieval system