KEGG   ENZYME: 1.3.8.9Help
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
BRITE hierarchy
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
Show
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 FAD as prosthetic group. 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
Fatty acid degradation
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)
MCC: 
714326(ACADVL)
RRO: 
104673335(ACADVL)
CJC: 
100399197(ACADVL)
MMU: 
11370(Acadvl)
RNO: 
25363(Acadvl)
CGE: 
NGI: 
103752159(Acadvl)
HGL: 
101707761(Acadvl)
OCU: 
100346654(ACADVL)
TUP: 
102486032(ACADVL)
CFA: 
489463(ACADVL)
AML: 
100472785(ACADVL)
UMR: 
103660348(ACADVL)
FCA: 
101094144(ACADVL)
PTG: 
102968012(ACADVL)
BTA: 
282130(ACADVL)
BOM: 
PHD: 
102343749(ACADVL)
CHX: 
102181987(ACADVL)
OAS: 
101117173(ACADVL)
SSC: 
100520636(ACADVL)
CFR: 
102516576(ACADVL)
BACU: 
103000307(ACADVL)
LVE: 
103077095(ACADVL)
ECB: 
100061583(ACADVL)
MYB: 
102257348(ACADVL)
MYD: 
102774563(ACADVL)
PALE: 
102879612(ACADVL)
MDO: 
100016619(ACADVL)
SHR: 
OAA: 
100089853(ACADVL)
AAM: 
106485823(ACADVL)
ASN: 
AMJ: 
102568802(ACADVL) 102576922(ACADVL)
PSS: 
CMY: 
102938508(ACADVL)
PBI: 
GJA: 
DRE: 
573723(acadvl)
TRU: 
101069830(acadvl)
MZE: 
101472773(acadvl)
OLA: 
101165349(acadvl)
XMA: 
102217028(acadvl)
LCM: 
102364546(ACADVL)
BFO: 
CIN: 
SPU: 
SKO: 
DME: 
DAN: 
DER: 
DPE: 
DSE: 
DSI: 
Dsimw501_GD11444(Dsim_GD11444)
DWI: 
DYA: 
Dyak_GE12038(Dyak_CG7461)
DGR: 
DMO: 
DVI: 
MDE: 
AGA: 
AAG: 
CQU: 
AME: 
412025(GB10312)
SOC: 
AEC: 
HST: 
CFO: 
NVI: 
TCA: 
BMOR: 
API: 
PHU: 
ISC: 
CEL: 
CELE_E04F6.5(acdh-12)
CBR: 
BMY: 
LOA: 
TSP: 
HRO: 
LGI: 
CRG: 
OBI: 
NVE: 
HMG: 
TAD: 
AQU: 
 » show all
Taxonomy
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 (1992) 1027-33.
  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 (1995) 2465-73.
  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 (2008) 9435-43.
  Sequence
[hsa:37]
Other DBs
ExplorEnz - The Enzyme Database: 
IUBMB Enzyme Nomenclature: 
ExPASy - ENZYME nomenclature database: 
BRENDA, the Enzyme Database: 

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