EzCatDB: T00009

DB codeT00009
CATH domainDomain 11.10.540.10
Domain 22.40.110.10
Domain 31.20.140.10Catalytic domain
E.C.1.3.8.4
CSA1ivh
MACiEM0068

CATH domainRelated DB codes (homologues)
1.10.540.10T00007,T00008
1.20.140.10T00007,T00008
2.40.110.10T00007,T00008

Enzyme Name
Swiss-protKEGG

P26440
Protein nameIsovaleryl-CoA dehydrogenase, mitochondrialisovaleryl-CoA dehydrogenase
isovaleryl-coenzyme A dehydrogenase
isovaleroyl-coenzyme A dehydrogenase
3-methylbutanoyl-CoA:(acceptor) oxidoreductase
SynonymsIVD
EC 1.3.99.10

KEGG pathways
MAP codePathways
MAP00280Valine, leucine and isoleucine degradation

Swiss-prot:Accession NumberP26440
Entry nameIVD_HUMAN
ActivityIsovaleryl-CoA + electron-transfer flavoprotein = 3-methylcrotonyl-CoA + reduced electron-transfer flavoprotein.
SubunitHomotetramer.
Subcellular locationMitochondrion matrix.
CofactorFAD.


CofactorsSubstratesProducts
KEGG-idC00016C02939C04253C03069C04570
CompoundFAD3-Methylbutanoyl-CoAElectron-transferring flavoprotein3-Methylbut-2-enoyl-CoAReduced electron-transferring flavoprotein
Typeamide group,amine group,aromatic ring (only carbon atom),aromatic ring (with nitrogen atoms),carbohydrate,nucleotideamide group,carbohydrate,nucleotide,peptide/protein,sulfide groupamide group,amine group,aromatic ring (only carbon atom),aromatic ring (with nitrogen atoms),carbohydrate,peptide/protein,phosphate group/phosphate ionamine group,carbohydrate,nucleotide,peptide/protein,sulfide groupamide group,amine group,aromatic ring (only carbon atom),aromatic ring (with nitrogen atoms),carbohydrate,peptide/protein,phosphate group/phosphate ion
1ivhA01UnboundUnboundUnboundUnboundUnbound
1ivhB01UnboundUnboundUnboundUnboundUnbound
1ivhC01UnboundUnboundUnboundUnboundUnbound
1ivhD01UnboundUnboundUnboundUnboundUnbound
1ivhA02Bound:FADUnboundUnboundUnboundUnbound
1ivhB02Bound:FADUnboundUnboundUnboundUnbound
1ivhC02Bound:FADUnboundUnboundUnboundUnbound
1ivhD02Bound:FADUnboundUnboundUnboundUnbound
1ivhA03UnboundAnalogue:COSUnboundUnboundUnbound
1ivhB03UnboundAnalogue:COSUnboundUnboundUnbound
1ivhC03UnboundAnalogue:COSUnboundUnboundUnbound
1ivhD03UnboundAnalogue:COSUnboundUnboundUnbound

Active-site residues
resource
Swiss-prot;P26440
pdbCatalytic residues
1ivhA01
1ivhB01
1ivhC01
1ivhD01
1ivhA02
1ivhB02
1ivhC02
1ivhD02
1ivhA03GLU 254
1ivhB03GLU 254
1ivhC03GLU 254
1ivhD03GLU 254

References for Catalytic Mechanism
ReferencesSectionsNo. of steps in catalysis
[3]Scheme 1, Scheme 4
[4]p.8463

references
[1]
CommentsACTIVE SITE GLU-283.
Medline ID95367542
PubMed ID7640268
JournalBiochemistry
Year1995
Volume34
Pages10146-52
AuthorsMohsen AW, Vockley J
TitleIdentification of the active site catalytic residue in human isovaleryl-CoA dehydrogenase.
Related Swiss-protP26440
[2]
PubMed ID8777688
JournalArch Dis Child Fetal Neonatal Ed
Year1996
Volume74
PagesF211-3
AuthorsLorek AK, Penrice JM, Cady EB, Leonard JV, Wyatt JS, Iles RA, Burns SP, Reynolds EO
TitleCerebral energy metabolism in isovaleric acidaemia.
[3]
PubMed ID9201918
JournalBiochemistry
Year1997
Volume36
Pages7761-8
AuthorsSchaller RA, Mohsen AW, Vockley J, Thorpe C
TitleMechanism-based inhibitor discrimination in the acyl-CoA dehydrogenases.
[4]
CommentsX-RAY CRYSTALLOGRAPHY (2.6 ANGSTROMS) OF 35-421.
Medline ID97361954
PubMed ID9214289
JournalBiochemistry
Year1997
Volume36
Pages8455-64
AuthorsTiffany KA, Roberts DL, Wang M, Paschke R, Mohsen AW, Vockley J, Kim JJ
TitleStructure of human isovaleryl-CoA dehydrogenase at 2.6 A resolution: structural basis for substrate specificity,.
Related PDB1ivh
Related Swiss-protP26440
[5]
PubMed ID9665741
JournalBiochemistry
Year1998
Volume37
Pages10325-35
AuthorsMohsen AW, Anderson BD, Volchenboum SL, Battaile KP, Tiffany K, Roberts D, Kim JJ, Vockley J
TitleCharacterization of molecular defects in isovaleryl-CoA dehydrogenase in patients with isovaleric acidemia.
[6]
PubMed ID10713113
JournalJ Biol Chem
Year2000
Volume275
Pages7958-63
AuthorsVolchenboum SL, Vockley J
TitleMitochondrial import and processing of wild type and type III mutant isovaleryl-CoA dehydrogenase.
[7]
PubMed ID11386848
JournalMol Genet Metab
Year2001
Volume73
Pages126-37
AuthorsMohsen AW, Navarette B, Vockley J
TitleIdentification of Caenorhabditis elegans isovaleryl-CoA dehydrogenase and structural comparison with other acyl-CoA dehydrogenases.
[8]
PubMed ID11592819
JournalMol Genet Metab
Year2001
Volume74
Pages226-37
AuthorsVolchenboum SL, Mohsen AW, Kim JJ, Vockley J
TitleArginine 387 of human isovaleryl-CoA dehydrogenase plays a crucial role in substrate/product binding.

comments
This enzyme belongs to the acyl-CoA dehydrogenase family.

createdupdated
2004-03-252012-10-03
Copyright: Nozomi Nagano, JST & CBRC-AIST
Funded by PRESTO/Japan Science and Technology Corporation (JST) (December 2001 - November 2004)
Funded by Grant-in-Aid for Publication of Scientific Research Results/Japan Society for the Promotion of Science (JSPS) (April 2005 - March 2006)
Funded by Grant-in-Aid for Scientific Research (B)/Japan Society for the Promotion of Science (JSPS) (April 2005 - March 2008)
Funded by BIRD/Japan Science and Technology Corporation (JST) (September 2005 - September 2010)
Funded by BIRD/Japan Science and Technology Corporation (JST) (October 2007 - September 2010)
Funded by Grant-in-Aid for Publication of Scientific Research Results/Japan Society for the Promotion of Science (JSPS) (April 2011 - March 2012)

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