KEGG ENZYME: 1.3.8.9

ENZYME: 1.3.8.9

Entry

EC 1.3.8.9                  Enzyme

Name

very-long-chain acyl-CoA dehydrogenase;
ACADVL (gene name).

Class

Oxidoreductases;
Acting on the CH-CH group of donors;
With a flavin as acceptor

Sysname

very-long-chain acyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase

Reaction(IUBMB)

a very-long-chain acyl-CoA + electron-transfer flavoprotein = a very-long-chain trans-2,3-dehydroacyl-CoA + reduced electron-transfer flavoprotein

Reaction(KEGG)

(other) R01279 R03990

Substrate

very-long-chain acyl-CoA [CPD:C20876];
electron-transfer flavoprotein [CPD:C04253]

Product

very-long-chain trans-2,3-dehydroacyl-CoA [CPD:C20879];
reduced electron-transfer flavoprotein [CPD:C04570]

Comment

Contains a tightly-bound FAD cofactor. One of several enzymes that catalyse the first step in fatty acids beta-oxidation. The enzyme is most active toward long-chain acyl-CoAs such as C14, C16 and C18, but is also active with very-long-chain acyl-CoAs up to 24 carbons. It shows no activity for substrates of less than 12 carbons. Its specific activity towards palmitoyl-CoA is more than 10-fold that of the long-chain acyl-CoA dehydrogenase [1]. cf. EC 1.3.8.1, short-chain acyl-CoA dehydrogenase, EC 1.3.8.7, medium-chain acyl-CoA dehydrogenase, and EC 1.3.8.8, long-chain acyl-CoA dehydrogenase.

History

EC 1.3.8.9 created 1961 as EC 1.3.2.2, transferred 1964 to EC 1.3.99.3, part transferred 2012 to EC 1.3.8.9

Pathway

ec00071

Fatty acid degradation

ec01100

Metabolic pathways

Orthology

K09479

very long chain acyl-CoA dehydrogenase

Genes

HSA:	37(ACADVL)

PTR:	455237(ACADVL)

PPS:	100986379(ACADVL)

GGO:	101143346(ACADVL)

PON:	100454381(ACADVL)

NLE:	100585921(ACADVL)

HMH:	116459317(ACADVL)

MCC:	714326(ACADVL)

MCF:	101925858(ACADVL)

MTHB:

126939682

MNI:	105473163(ACADVL)

CSAB:

103242278(ACADVL)

CATY:

105582769(ACADVL)

PANU:

101018882(ACADVL)

TGE:	112608977(ACADVL)

MLEU:

105549907(ACADVL)

RRO:	104673335(ACADVL)

RBB:	108514298(ACADVL)

TFN:	117067801(ACADVL)

PTEH:

111521283(ACADVL)

CANG:

105523588(ACADVL)

CJC:	100399197(ACADVL)

SBQ:	101028298(ACADVL)

CIMI:

108306602(ACADVL)

CSYR:

103263239(ACADVL)

MMUR:

105877978(ACADVL)

LCAT:

123650853(ACADVL)

PCOQ:

105819433(ACADVL)

OGA:	100947350(ACADVL)

MMU:	11370(Acadvl)

MCAL:

110304163(Acadvl)

MPAH:

110332221(Acadvl)

RNO:	25363(Acadvl)

MCOC:

116079063(Acadvl)

ANU:	117711753(Acadvl)

MUN:	110555097(Acadvl)

CGE:	100767527(Acadvl)

MAUA:

101824933(Acadvl)

PROB:

127219014(Acadvl)

PLEU:

114693210(Acadvl)

MORG:

121456541(Acadvl)

MFOT:

126513897

AAMP:

119811176(Acadvl)

NGI:	103752159(Acadvl)

HGL:	101707761(Acadvl)

CPOC:

100713909(Acadvl)

CCAN:

109674520(Acadvl)

DORD:

105983460(Acadvl)

DSP:	122100421(Acadvl)

PLOP:

125365206(Acadvl)

NCAR:

124981031

MMMA:

107157970(Acadvl)

OCU:

100346654

OPI:	101517023(ACADVL)

TUP:	102486032(ACADVL)

GVR:	103591441(ACADVL)

CFA:	489463(ACADVL)

CLUD:

112647487(ACADVL)

VVP:	112924990(ACADVL)

VLG:	121499762(ACADVL)

NPO:	129496982(ACADVL)

AML:	100472785(ACADVL)

UMR:	103660348(ACADVL)

UAH:	113271108(ACADVL)

UAR:	123792174(ACADVL)

ELK:

111161073

LLV:

125087809

MPUF:

101679496(ACADVL)

MNP:	132003338(ACADVL)

MLK:	131815752(ACADVL)

NVS:	122906685(ACADVL)

ORO:	101376612(ACADVL)

EJU:	114225362(ACADVL)

ZCA:	113939372(ACADVL)

MLX:	117999991(ACADVL)

NSU:	110584452(ACADVL)

LWW:	102738302(ACADVL)

FCA:	101094144(ACADVL)

PYU:	121016030(ACADVL)

PCOO:

112856982

PBG:	122484852(ACADVL)

PVIV:

125151822(ACADVL)

LRUF:

124519482

PTG:	102968012(ACADVL)

PPAD:

109245981(ACADVL)

PUC:

125927068

AJU:

106972414

HHV:	120231442(ACADVL)

BTA:	282130(ACADVL)

BOM:

102273950

BIU:	109573934(ACADVL)

BBUB:

102405745(ACADVL)

BBIS:

104993930(ACADVL)

CHX:	102181987(ACADVL)

OAS:	101117173(ACADVL)

BTAX:

128064846(ACADVL)

ODA:	120866080(ACADVL)

CCAD:

122446217(ACADVL)

MBEZ:

129542624(ACADVL)

SSC:	100520636(ACADVL)

CFR:	102516576(ACADVL)

CBAI:

105077481(ACADVL)

CDK:	105103048(ACADVL)

VPC:	102539565(ACADVL)

BACU:

103000307(ACADVL)

BMUS:

118886462(ACADVL)

LVE:	103077095(ACADVL)

OOR:	101275668(ACADVL)

DLE:	111185665(ACADVL)

PCAD:

102973774(ACADVL)

PSIU:

116745056(ACADVL)

NASI:

112390407(ACADVL)

ECB:	100061583(ACADVL)

EPZ:	103557744(ACADVL)

EAI:	106844174(ACADVL)

MYB:	102257348(ACADVL)

MYD:	102774563(ACADVL)

MMYO:

118672042(ACADVL)

MLF:	102423177(ACADVL)

PKL:	118726931(ACADVL)

EFUS:

103296934(ACADVL)

MNA:	107544584(ACADVL)

DRO:	112308631(ACADVL)

SHON:

118974685(ACADVL)

AJM:	119037137(ACADVL)

PDIC:

114514993(ACADVL)

PHAS:

123809242(ACADVL)

MMF:	118633802(ACADVL)

PPAM:

129072309(ACADVL)

HAI:	109394616(ACADVL)

RFQ:	117012846(ACADVL)

PALE:

102879612(ACADVL)

PGIG:

120589632(ACADVL)

PVP:	105291734(ACADVL)

RAY:	107509515(ACADVL)

MJV:	108401220(ACADVL)

TOD:	119232659(ACADVL)

SARA:

101545206(ACADVL)

LAV:	100657276(ACADVL)

TMU:

101361348

ETF:	101645138(ACADVL)

DNM:	101438130(ACADVL)

MDO:	100016619(ACADVL) 100032148

GAS:	123242000 123244462(ACADVL)

SHR:	100925698 100931501(ACADVL)

AFZ:	127558293 127560911

PCW:	110193114 110194404(ACADVL)

OAA:	100089853(ACADVL)

LSR:	110482134(ACADVL)

CCAE:

111922435(ACADVL)

SVG:	106864280(ACADVL)

ACHC:

115338360(ACADVL)

AGEN:

126034670

AAM:	106485823(ACADVL)

AROW:

112962139(ACADVL) 112963323

NPD:	112947461(ACADVL)

TGT:	104573439(ACADVL)

DNE:	112981697(ACADVL)

SCAM:

104153221(ACADVL)

ASN:	102371669 102384902(ACADVL)

AMJ:	102568802(ACADVL) 102576922

CPOO:

109308570(ACADVL)

GGN:	109288127(ACADVL)

PSS:	102443861(ACADVL) 102456803

CMY:	102938508(ACADVL)

CCAY:

125628616(ACADVL) 125642751

DCC:	119849363 119861618 122457698

CPIC:

101946768 101948805(ACADVL)

TST:	117883233(ACADVL)

CABI:

116828964 116839188(ACADVL)

MRV:	120372442 120381766(ACADVL)

ASAO:

132777544(ACADVL)

PVT:	110082312(ACADVL)

SUND:

121933466(ACADVL)

PBI:	103051941 103059781(ACADVL)

PMUR:

107291707 107295343(ACADVL)

CTIG:

120305692(ACADVL) 120307167

TSR:	106542417(ACADVL) 106551584

PGUT:

117656436 117659250 117669843

APRI:

131196872(ACADVL) 131200568

PTEX:

113450919(ACADVL)

NSS:	113425170(ACADVL)

VKO:	123016648(ACADVL)

PMUA:

114581599(ACADVL) 114598214

PRAF:

128400158(ACADVL) 128414927

ZVI:	118093983 118094793(ACADVL)

HCG:	128331405(ACADVL) 128349410

GJA:	107118083(ACADVL) 107125630

STOW:

125445140(ACADVL)

EMC:	129331940 129340369(ACADVL)

XLA:	108711808(acadvl.L)

XTR:	100216267(acadvl)

RTEM:

120921798(ACADVL)

BBUF:

120986711(ACADVL)

BGAR:

122922346(ACADVL)

MUO:	115457771(ACADVL)

GSH:	117350647(ACADVL)

DRE:	573723(acadvl)

SRX:	107731915 107738021

SANH:

107675499 107683542(acadvl)

SGH:	107595296 107597078

CCAR:

109073618 109092601

CAUA:

113052224 113105619

CGIB:

127961630 128017217

PTET:

122349779(acadvl)

LROH:

127167632(acadvl)

OMC:	131543239(acadvl)

PPRM:

120469949(acadvl)

RKG:	130091451(acadvl)

MAMB:

125265528(acadvl)

CIDE:

127515814

MASI:

127415437 127417077 127433914

TROS:

130545585 130546066(acadvl) 130550983

TDW:	130417368(acadvl) 130419068

MANU:

129438883(acadvl)

IPU:

108267840

IFU:	128610167(acadvl)

PHYP:

113540141(acadvl)

SMEO:

124387396(acadvl)

TFD:	113654726(acadvl)

TVC:	132848746(acadvl)

AMEX:

103045531(acadvl)

CMAO:

118826677(acadvl)

EEE:	113567683(acadvl)

CHAR:

105906682 105907142(acadvl)

TRU:	101069830(acadvl)

TFS:	130522023(acadvl)

TNG:	GSTEN00019248G001

LCO:	104937817(acadvl)

NCC:

104942951

TBEN:

117488099(acadvl)

CGOB:

115023469(acadvl)

PGEO:

117463629(acadvl)

GACU:

117552716(acadvl)

EMAC:

134876077(acadvl)

ELY:	117246106(acadvl)

EFO:	125883637(acadvl)

SLUC:

116048989(acadvl)

ECRA:

117934572(acadvl)

ESP:	116686337(acadvl)

PFLV:

114545512(acadvl)

GAT:	120821571(acadvl)

PPUG:

119222335(acadvl)

AFB:	129093499(acadvl)

CLUM:

117747431(acadvl)

PSWI:

130211767(acadvl)

MSAM:

119911752(acadvl)

SCHU:

122870721(acadvl)

CUD:	121504728(acadvl)

ALAT:

119026810(acadvl)

MZE:	101472773(acadvl)

ONL:	100703037(acadvl)

OAU:	116330727(acadvl)

OLA:	101165349(acadvl)

OML:	112156048(acadvl)

CSAI:

133420004(acadvl)

XMA:	102217028(acadvl)

XCO:	114157390(acadvl)

XHE:	116732534(acadvl)

PRET:

103480485(acadvl)

PFOR:

103137528(acadvl)

PLAI:

106945534(acadvl)

PMEI:

106906747(acadvl)

GAF:	122819841(acadvl)

PPRL:

129379427(acadvl)

CVG:	107087781(acadvl)

CTUL:

119789781(acadvl)

GMU:	124880174(acadvl)

NFU:	107372430(acadvl)

KMR:	108241814(acadvl)

ALIM:

106529201(acadvl)

NWH:	119416326(acadvl)

AOCE:

111575585(acadvl)

MCEP:

124997830(acadvl)

CSEM:

103386901(acadvl)

POV:	109634887(acadvl)

SSEN:

122766124(acadvl)

HHIP:

117752053(acadvl)

HSP:	118102302(acadvl)

SMAU:

118317852(acadvl)

LCF:

108897054

SDU:	111232992(acadvl)

SLAL:

111665745(acadvl)

XGL:	120797419(acadvl)

HCQ:	109506947(acadvl)

SSCV:

125981931

SBIA:

133494776(acadvl)

PEE:	133397629(acadvl)

PTAO:

133472714(acadvl)

BPEC:

110157677(acadvl)

MALB:

109966474(acadvl)

BSPL:

114843947(acadvl)

SJO:	128383753(acadvl)

SASA:

106602820(acadvl)

STRU:

115155021(acadvl)

OTW:	112216747(acadvl)

OMY:	110490130(acadvl)

OGO:	123994754(acadvl)

ONE:	115106836(acadvl)

OKI:	109864415(acadvl)

OKE:	118365919(acadvl)

SALP:

111950426(acadvl)

SNH:	120057400(acadvl)

CCLU:

121579727(acadvl)

ELS:	105010800(acadvl)

SFM:	108918781(acadvl) 108923151

PKI:	111860921(acadvl)

AANG:

118222760(acadvl)

LOC:	102693936(acadvl)

PSPA:

121306545(acadvl)

ARUT:

117404600 117967919

PSEX:

120524994(acadvl)

LCM:	102364546(ACADVL)

RTP:	109918899(acadvl)

CPLA:

122544279(acadvl) 122556076

HOC:	132821718 132835154

LERI:

129694004(acadvl) 129703268

PMRN:

116944603(ACADVL)

LRJ:	133340840(ACADVL)

BFO:

118427578

BBEL:

109463957

CIN:

100183990

SCLV:

120328435

SPU:

584710

LPIC:

129279127

APLC:

110981027

AJC:

117106771

SKO:

100369524

DME:	Dmel_CG7461(Acadvl)

DER:

6547331

DSE:

6609917

DSI:	Dsimw501_GD11444(Dsim_GD11444)

DYA:	Dyak_GE12038

DAN:

6506293

DSR:

110177854

DPO:

26532094

DPE:

6602657

DMN:

117188910

DGR:

6557587

DMO:	Dmoj_GI12099

DAZ:

108614533

DNV:

108651701

DHE:

111592309

DVI:

6623490

CCAT:

101461538

BOD:

106622331

BDR:

105223246

RZE:	108354643 108361541 108378210

AOQ:

129247130

TDA:

119687575

MDE:

101891231

SCAC:

106091613

LCQ:	111683083 111683088 111683092

LSQ:	119608560 119608561 119608567

GFS:

119642989

ECOE:

129938849

CLON:

129610657

HIS:

119651165

AGA:	1275626 133393306 133394118 133394119

ACOZ:

120958362 120958363 120958364 120958365

AARA:

120901834 120902053 120902103 120904618

AMER:

121597151 121597152 121597153 121597155

ASTE:

118511710 118511711 118511712 118511713

AFUN:

125766327 125766329 125766330 125766331

AMOU:

128302062 128302063 128304425 128305932

AALI:

118462065 118462066 118462067

AAG:	23687873 5565366 5565367

AALB:

109400327 109400349 109425168 109425169

CQU:	CpipJ_CPIJ016451 CpipJ_CPIJ016452 CpipJ_CPIJ016453 CpipJ_CPIJ016454

CPII:

120417357 120417361 120417378

CNS:

116336875

BCOO:

119085955

AME:

412025

ACER:

107993296

ALAB:

122713268

ADR:

102672182

AFLR:

100865647

BIM:

100747671

BBIF:

117207816

BVK:

117238208

BVAN:

117160011

BTER:

100651720

BAFF:

126919937

BPYO:

122570882

BPAS:

132906335

FVI:

122531524

CCAL:

108632159

OBB:

114874036

OLG:

117606311

MGEN:

117225101

NMEA:

116431343

CGIG:

122404056

SOC:

105200554

MPHA:

105838446

AEC:

105149521

ACEP:

105622411

PBAR:

105429882

VEM:

105562928

HST:

105188087

DQU:

106746266

CFO:

105251587

FEX:

115241390

LHU:

105667331

PGC:

109854598

OBO:

105276471

PCF:

106788263

PFUC:

122517614

VPS:

122637938

VCRB:

124432788

VVE:

124947267

NVI:

100116828

CSOL:

105368265

TPRE:

106657066

LHT:

122497687

LBD:

127286425

MDL:

103574466

CGLO:

123261711

FAS:

105263418

DAM:

107047818

AGIF:

122849565 122855670

CINS:

118069543

VCAN:

122415786

CCIN:

107274922

DSM:

124411841

NPT:

124218768

NFB:

124183961

NLO:

107223127

NVG:

124306365

AROA:

105683850

TCA:

659957

DPA:

109541041

SOY:

115876810

AGRG:

126748220

ATD:

109601372

CSET:

123317940

AGB:

108910959

NVL:	108562362 108569494

APLN:

108741828

PPYR:

116160986

OTU:

111425163

BMOR:

101744941

BMAN:

114241040

MSEX:

115450554 119192036

DPL:	KGM_210602

BANY:

112050744

MJU:

123877800

NIQ:

126771213

VCD:

124543694

MCIX:

123663235

PMAC:

106710891

PXU:

106124808

PRAP:

110996061

PBX:

123714584

PNAP:

125059555

ZCE:

119829410

CCRC:

123702246

LSIN:

126976352

HZE:

124630404

TNL:

113500974

SLIU:

111353597

OFU:

114364297

PGW:

126373682

CFEL:

113381136

CCRN:

123302345

API:

100162051

DNX:

107164135

AGS:	114127836 114127838

RMD:

113560282

ACOO:

126835221

DVT:

126910520

BTAB:

109036499

DCI:

103511783

CLEC:

106668658

HHAL:

106691567

NLU:

111057385

HVI:

124362786

MQU:

128990136

PHU:	Phum_PHUM617730

FOC:

113203933

ZNE:

110832869

CSEC:

111868786

SGRE:

126351366

BROR:

134529049

IEL:

124153573

FCD:

110843829

DPX:	DAPPUDRAFT_327246

DMK:

116922047

DPZ:

124336955

PVM:	113813193 113823665

PJA:

122259512

PCHN:

125031151

PMOO:

119580640

HAME:

121878582

PCLA:

123762221

CQD:

128697150

PTRU:

123513027

ESN:

126982288

HAZT:

108669783

EAF:

111695316

LSM:	121122352 121126155

TCF:

131890658

PPOI:

119109527

DSV:

119439895

RSAN:

119386460

RMP:

119178100

VDE:

111251465

VJA:

111270259

TUT:

107362076

DPTE:

113799491

DFR:

124496042

CSCU:

111630174

PTEP:

107450301

ABRU:

129988380

UDV:	129219424 129232459

SDM:	118184637 118199840

LPOL:

106465874

PMEO:

129599287

CEL:	CELE_E04F6.5(acdh-12)

CBR:

CBG_13088

CRQ:	GCK72_006440

NAI:	NECAME_17750

BMY:	BM_BM3112(Bma-acdh-12)

LOA:	LOAG_00695

TSP:

Tsp_10283

HRO:	HELRODRAFT_186034

LGI:	LOTGIDRAFT_177715

PVUL:

126815130

PCAN:

112555772

BGT:

106055817

GAE:

121375144

HRF:

124145143

HRJ:

124282755

CRG:

105344745

CVN:

111135491

CANU:

128181060

OED:	125659500 125667826

MYI:

110463202

PMAX:

117342228

MCAF:

127737844

MMER:

123553917

RPHI:

132750528

DPOL:

127834180

OBI:

106875659

OSN:

115209819

LAK:

106158444

OVI:	T265_06522

NVE:

5510970

EPA:

110251159

ATEN:

116295518

ADF:

107343075

AMIL:

114953943

PDAM:

113681975

SPIS:

111335626

DGT:

114521443

XEN:

124446405

HMG:

100201063

HSY:

130636588

TAD:	TRIADDRAFT_20833 TRIADDRAFT_23854 TRIADDRAFT_30874 TRIADDRAFT_31731 TRIADDRAFT_31757 TRIADDRAFT_31810 TRIADDRAFT_34622

AQU:

100638454

GAU:

GAU_0440

» show all

Reference

1 [PMID:1730632]

Authors

Izai K, Uchida Y, Orii T, Yamamoto S, Hashimoto T

Title

Novel fatty acid beta-oxidation enzymes in rat liver mitochondria. I. Purification and properties of very-long-chain acyl-coenzyme A dehydrogenase.

Journal

J Biol Chem 267:1027-33 (1992)

Sequence

[rno:25363]

Reference

2 [PMID:7769092]

Authors

Aoyama T, Souri M, Ushikubo S, Kamijo T, Yamaguchi S, Kelley RI, Rhead WJ, Uetake K, Tanaka K, Hashimoto T

Title

Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients.

Journal

J Clin Invest 95:2465-73 (1995)
DOI:10.1172/JCI117947

Sequence

[hsa:37]

Reference

3 [PMID:18227065]

Authors

McAndrew RP, Wang Y, Mohsen AW, He M, Vockley J, Kim JJ

Title

Structural basis for substrate fatty acyl chain specificity: crystal structure of human very-long-chain acyl-CoA dehydrogenase.

Journal

J Biol Chem 283:9435-43 (2008)
DOI:10.1074/jbc.M709135200

Sequence

[hsa:37]

Other DBs

ExplorEnz - The Enzyme Database:

1.3.8.9

IUBMB Enzyme Nomenclature:

1.3.8.9

ExPASy - ENZYME nomenclature database:

1.3.8.9

BRENDA, the Enzyme Database:

1.3.8.9

DBGET integrated database retrieval system