T00009
type: Enzyme
cath: Butyryl-Coa Dehydrogenase, subunit A; domain 1
cath: Butyryl-CoA Dehydrogenase, subunit A; domain 2
cath: Butyryl-CoA Dehydrogenase, subunit A; domain 3
ec: https://purl.uniprot.org/enzyme/1.3.8.4
csa: 1ivh
macie: M0068
uniprot: P26440
kegg enzyme: isovaleryl-CoA dehydrogenase
kegg enzyme: isovaleryl-coenzyme A dehydrogenase
kegg enzyme: isovaleroyl-coenzyme A dehydrogenase
kegg enzyme: 3-methylbutanoyl-CoA:(acceptor) oxidoreductase
kegg pathway: Valine, leucine and isoleucine degradation
kegg cofactor: FAD
kegg substrate: 3-Methylbutanoyl-CoA
kegg substrate: Electron-transferring flavoprotein
kegg product: 3-Methylbut-2-enoyl-CoA
kegg product: Reduced electron-transferring flavoprotein
pdb: 1ivhA01
pdb: 1ivhB01
pdb: 1ivhC01
pdb: 1ivhD01
pdb: 1ivhA02
pdb: 1ivhB02
pdb: 1ivhC02
pdb: 1ivhD02
pdb: 1ivhA03
pdb: 1ivhB03
pdb: 1ivhC03
pdb: 1ivhD03
pdb bound state: Unbound
  compound: 3-Methylbutanoyl-CoA
  pdb: 1ivhA01
pdb bound state: Unbound
  compound: 3-Methylbutanoyl-CoA
  pdb: 1ivhB01
pdb bound state: Unbound
  compound: 3-Methylbutanoyl-CoA
  pdb: 1ivhC01
pdb bound state: Unbound
  compound: 3-Methylbutanoyl-CoA
  pdb: 1ivhD01
pdb bound state: Unbound
  compound: 3-Methylbutanoyl-CoA
  pdb: 1ivhA02
pdb bound state: Unbound
  compound: 3-Methylbutanoyl-CoA
  pdb: 1ivhB02
pdb bound state: Unbound
  compound: 3-Methylbutanoyl-CoA
  pdb: 1ivhC02
pdb bound state: Unbound
  compound: 3-Methylbutanoyl-CoA
  pdb: 1ivhD02
pdb bound state: Analogue
  compound: 3-Methylbutanoyl-CoA
  pdb: 1ivhA03
  ligand het code: COS
pdb bound state: Analogue
  compound: 3-Methylbutanoyl-CoA
  pdb: 1ivhB03
  ligand het code: COS
pdb bound state: Analogue
  compound: 3-Methylbutanoyl-CoA
  pdb: 1ivhC03
  ligand het code: COS
pdb bound state: Analogue
  compound: 3-Methylbutanoyl-CoA
  pdb: 1ivhD03
  ligand het code: COS
pdb bound state: Unbound
  compound: Electron-transferring flavoprotein
  pdb: 1ivhA01
pdb bound state: Unbound
  compound: Electron-transferring flavoprotein
  pdb: 1ivhB01
pdb bound state: Unbound
  compound: Electron-transferring flavoprotein
  pdb: 1ivhC01
pdb bound state: Unbound
  compound: Electron-transferring flavoprotein
  pdb: 1ivhD01
pdb bound state: Unbound
  compound: Electron-transferring flavoprotein
  pdb: 1ivhA02
pdb bound state: Unbound
  compound: Electron-transferring flavoprotein
  pdb: 1ivhB02
pdb bound state: Unbound
  compound: Electron-transferring flavoprotein
  pdb: 1ivhC02
pdb bound state: Unbound
  compound: Electron-transferring flavoprotein
  pdb: 1ivhD02
pdb bound state: Unbound
  compound: Electron-transferring flavoprotein
  pdb: 1ivhA03
pdb bound state: Unbound
  compound: Electron-transferring flavoprotein
  pdb: 1ivhB03
pdb bound state: Unbound
  compound: Electron-transferring flavoprotein
  pdb: 1ivhC03
pdb bound state: Unbound
  compound: Electron-transferring flavoprotein
  pdb: 1ivhD03
pdb bound state: Unbound
  compound: FAD
  pdb: 1ivhA01
pdb bound state: Unbound
  compound: FAD
  pdb: 1ivhB01
pdb bound state: Unbound
  compound: FAD
  pdb: 1ivhC01
pdb bound state: Unbound
  compound: FAD
  pdb: 1ivhD01
pdb bound state: Unbound
  compound: FAD
  pdb: 1ivhA03
pdb bound state: Unbound
  compound: FAD
  pdb: 1ivhB03
pdb bound state: Unbound
  compound: FAD
  pdb: 1ivhC03
pdb bound state: Unbound
  compound: FAD
  pdb: 1ivhD03
pdb bound state: Unbound
  compound: 3-Methylbut-2-enoyl-CoA
  pdb: 1ivhA01
pdb bound state: Unbound
  compound: 3-Methylbut-2-enoyl-CoA
  pdb: 1ivhB01
pdb bound state: Unbound
  compound: 3-Methylbut-2-enoyl-CoA
  pdb: 1ivhC01
pdb bound state: Unbound
  compound: 3-Methylbut-2-enoyl-CoA
  pdb: 1ivhD01
pdb bound state: Unbound
  compound: 3-Methylbut-2-enoyl-CoA
  pdb: 1ivhA02
pdb bound state: Unbound
  compound: 3-Methylbut-2-enoyl-CoA
  pdb: 1ivhB02
pdb bound state: Unbound
  compound: 3-Methylbut-2-enoyl-CoA
  pdb: 1ivhC02
pdb bound state: Unbound
  compound: 3-Methylbut-2-enoyl-CoA
  pdb: 1ivhD02
active site residues resources: P26440
references: Identification of the active site catalytic residue in human isovaleryl-CoA dehydrogenase.
references: Cerebral energy metabolism in isovaleric acidaemia.
references: Mechanism-based inhibitor discrimination in the acyl-CoA dehydrogenases.
references: Structure of human isovaleryl-CoA dehydrogenase at 2.6 A resolution: structural basis for substrate specificity,.
references: Characterization of molecular defects in isovaleryl-CoA dehydrogenase in patients with isovaleric acidemia.
references: Mitochondrial import and processing of wild type and type III mutant isovaleryl-CoA dehydrogenase.
references: Identification of Caenorhabditis elegans isovaleryl-CoA dehydrogenase and structural comparison with other acyl-CoA dehydrogenases.
references: Arginine 387 of human isovaleryl-CoA dehydrogenase plays a crucial role in substrate/product binding.
comment: This enzyme belongs to the acyl-CoA dehydrogenase family.

Butyryl-Coa Dehydrogenase, subunit A; domain 1
type: Cath
references: http://www.cathdb.info/version/latest/superfamily/1.10.540.10

Butyryl-CoA Dehydrogenase, subunit A; domain 2
type: Cath
references: http://www.cathdb.info/version/latest/superfamily/2.40.110.10

Butyryl-CoA Dehydrogenase, subunit A; domain 3
type: Cath
references: http://www.cathdb.info/version/latest/superfamily/1.20.140.10
catalytic domain: index.html

Valine, leucine and isoleucine degradation
type: KeggPathway
references: KEGG:MAP00280

FAD
type: KeggCofactor
references: KEGG:C00016
compound type: amide group
compound type: amine group
compound type: aromatic ring (only carbon atom)
compound type: aromatic ring (with nitrogen atoms)
compound type: carbohydrate
compound type: nucleotide
references: CHEBI:16238
references: http://pubchem.ncbi.nlm.nih.gov/summary/summary.cgi

3-Methylbutanoyl-CoA
type: KeggSubstrate
references: KEGG:C02939
compound type: amide group
compound type: carbohydrate
compound type: nucleotide
compound type: peptide/protein
compound type: sulfide group
references: CHEBI:15487
references: http://pubchem.ncbi.nlm.nih.gov/summary/summary.cgi

Electron-transferring flavoprotein
type: KeggSubstrate
references: KEGG:C04253
compound type: amide group
compound type: amine group
compound type: aromatic ring (only carbon atom)
compound type: aromatic ring (with nitrogen atoms)
compound type: carbohydrate
compound type: peptide/protein
compound type: phosphate group/phosphate ion

3-Methylbut-2-enoyl-CoA
type: KeggProduct
references: KEGG:C03069
compound type: amine group
compound type: carbohydrate
compound type: nucleotide
compound type: peptide/protein
compound type: sulfide group
references: CHEBI:15486
references: http://pubchem.ncbi.nlm.nih.gov/summary/summary.cgi

Reduced electron-transferring flavoprotein
type: KeggProduct
references: KEGG:C04570
compound type: amide group
compound type: amine group
compound type: aromatic ring (only carbon atom)
compound type: aromatic ring (with nitrogen atoms)
compound type: carbohydrate
compound type: peptide/protein
compound type: phosphate group/phosphate ion

1ivhA03
type: Pdb
references: 1ivh
references: http://www.ebi.ac.uk/thornton-srv/databases/cgi-bin/pdbsum/GetPage.pl
references: PDB:1ivh
catalytic residue: GLU 254

1ivhB03
type: Pdb
references: 1ivh
references: http://www.ebi.ac.uk/thornton-srv/databases/cgi-bin/pdbsum/GetPage.pl
references: PDB:1ivh
catalytic residue: GLU 254

1ivhC03
type: Pdb
references: 1ivh
references: http://www.ebi.ac.uk/thornton-srv/databases/cgi-bin/pdbsum/GetPage.pl
references: PDB:1ivh
catalytic residue: GLU 254

1ivhD03
type: Pdb
references: 1ivh
references: http://www.ebi.ac.uk/thornton-srv/databases/cgi-bin/pdbsum/GetPage.pl
references: PDB:1ivh
catalytic residue: GLU 254

Identification of the active site catalytic residue in human isovaleryl-CoA dehydrogenase.
type: BibliographicResource
comment: ACTIVE SITE GLU-283.
medline id: 95367542
references: http://www.ncbi.nlm.nih.gov/pubmed/7640268
journal: Biochemistry
volume: 34
pages: 10146-52
published year: 1995
authors:
  item: Mohsen AW
  item: Vockley J
references: P26440

Cerebral energy metabolism in isovaleric acidaemia.
type: BibliographicResource
references: http://www.ncbi.nlm.nih.gov/pubmed/8777688
journal: Arch Dis Child Fetal Neonatal Ed
volume: 74
pages: F211-3
published year: 1996
authors:
  item: Lorek AK
  item: Penrice JM
  item: Cady EB
  item: Leonard JV
  item: Wyatt JS
  item: Iles RA
  item: Burns SP
  item: Reynolds EO

Mechanism-based inhibitor discrimination in the acyl-CoA dehydrogenases.
type: BibliographicResource
references: http://www.ncbi.nlm.nih.gov/pubmed/9201918
journal: Biochemistry
volume: 36
pages: 7761-8
published year: 1997
authors:
  item: Schaller RA
  item: Mohsen AW
  item: Vockley J
  item: Thorpe C

Structure of human isovaleryl-CoA dehydrogenase at 2.6 A resolution: structural basis for substrate specificity,.
type: BibliographicResource
comment: X-RAY CRYSTALLOGRAPHY (2.6 ANGSTROMS) OF 35-421.
medline id: 97361954
references: http://www.ncbi.nlm.nih.gov/pubmed/9214289
journal: Biochemistry
volume: 36
pages: 8455-64
published year: 1997
authors:
  item: Tiffany KA
  item: Roberts DL
  item: Wang M
  item: Paschke R
  item: Mohsen AW
  item: Vockley J
  item: Kim JJ
references: http://www.ebi.ac.uk/thornton-srv/databases/cgi-bin/pdbsum/GetPage.pl
references: P26440

Characterization of molecular defects in isovaleryl-CoA dehydrogenase in patients with isovaleric acidemia.
type: BibliographicResource
references: http://www.ncbi.nlm.nih.gov/pubmed/9665741
journal: Biochemistry
volume: 37
pages: 10325-35
published year: 1998
authors:
  item: Mohsen AW
  item: Anderson BD
  item: Volchenboum SL
  item: Battaile KP
  item: Tiffany K
  item: Roberts D
  item: Kim JJ
  item: Vockley J

Mitochondrial import and processing of wild type and type III mutant isovaleryl-CoA dehydrogenase.
type: BibliographicResource
references: http://www.ncbi.nlm.nih.gov/pubmed/10713113
journal: J Biol Chem
volume: 275
pages: 7958-63
published year: 2000
authors:
  item: Volchenboum SL
  item: Vockley J

Identification of Caenorhabditis elegans isovaleryl-CoA dehydrogenase and structural comparison with other acyl-CoA dehydrogenases.
type: BibliographicResource
references: http://www.ncbi.nlm.nih.gov/pubmed/11386848
journal: Mol Genet Metab
volume: 73
pages: 126-37
published year: 2001
authors:
  item: Mohsen AW
  item: Navarette B
  item: Vockley J

Arginine 387 of human isovaleryl-CoA dehydrogenase plays a crucial role in substrate/product binding.
type: BibliographicResource
references: http://www.ncbi.nlm.nih.gov/pubmed/11592819
journal: Mol Genet Metab
volume: 74
pages: 226-37
published year: 2001
authors:
  item: Volchenboum SL
  item: Mohsen AW
  item: Kim JJ
  item: Vockley J

ReferenceForCatalyticMechanism
reference index: 3
reference section: Scheme 1, Scheme 4

ReferenceForCatalyticMechanism
reference index: 4
reference section: p.8463