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STRUCTURE OF HUMAN ISOVALERYL-COA DEHYDROGENASE AT 2.6 ANGSTROMS RESOLUTION: STRUCTURAL BASIS FOR SUBSTRATE SPECIFICITY

Source organism: Homo sapiens
Biochemical function: flavin adenine dinucleotide binding
Biological process: fatty acid beta-oxidation using acyl-CoA dehydrogenase
Cellular component: mitochondrial matrix

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EC 1.3.8.4: Isovaleryl-CoA dehydrogenase

Reaction catalysed:
Isovaleryl-CoA + electron-transfer flavoprotein = 3-methylcrotonyl-CoA + reduced electron-transfer flavoprotein
Systematic name:
3-methylbutanoyl-CoA:electron-transfer flavoprotein oxidoreductase
Alternative Name(s):
  • 3-methylbutanoyl-CoA:(acceptor) oxidoreductase
  • Isovaleroyl-coenzyme A dehydrogenase
  • Isovaleryl-coenzyme A dehydrogenase

EC 1.3.8.1: Short-chain acyl-CoA dehydrogenase

Reaction catalysed:
A short-chain acyl-CoA + electron-transfer flavoprotein = a short-chain trans-2,3-dehydroacyl-CoA + reduced electron-transfer flavoprotein
Systematic name:
Short-chain acyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase
Alternative Name(s):
  • 3-hydroxyacyl CoA reductase
  • ACADS (gene name)
  • Butanoyl-CoA dehydrogenase
  • Butanoyl-CoA:(acceptor) 2,3-oxidoreductase
  • Butyryl coenzyme A dehydrogenase
  • Butyryl dehydrogenase
  • Butyryl-CoA dehydrogenase
  • Enoyl-coenzyme A reductase
  • Ethylene reductase
  • Short-chain acyl CoA dehydrogenase
  • Short-chain acyl-coenzyme A dehydrogenase
  • Unsaturated acyl coenzyme A reductase
  • Unsaturated acyl-CoA reductase

GO terms

Sequence families

Pfam Protein families (Pfam)
PF08028
Domain description: Acyl-CoA dehydrogenase, C-terminal domain
Occurring in:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial

PF00441
Domain description: Acyl-CoA dehydrogenase, C-terminal domain
Occurring in:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial
The deposited structure of PDB entry 1ivh contains 4 copies of Pfam domain PF00441 (Acyl-CoA dehydrogenase, C-terminal domain) in Isovaleryl-CoA dehydrogenase, mitochondrial. Showing 1 copy in chain A.

PF02770
Domain description: Acyl-CoA dehydrogenase, middle domain
Occurring in:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial
The deposited structure of PDB entry 1ivh contains 4 copies of Pfam domain PF02770 (Acyl-CoA dehydrogenase, middle domain) in Isovaleryl-CoA dehydrogenase, mitochondrial. Showing 1 copy in chain A.

PF02771
Domain description: Acyl-CoA dehydrogenase, N-terminal domain
Occurring in:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial
The deposited structure of PDB entry 1ivh contains 4 copies of Pfam domain PF02771 (Acyl-CoA dehydrogenase, N-terminal domain) in Isovaleryl-CoA dehydrogenase, mitochondrial. Showing 1 copy in chain A.

InterPro InterPro annotations
IPR006089
Domain description: Acyl-CoA dehydrogenase, conserved site
Occurring in:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial
IPR009075
Domain description: Acyl-CoA dehydrogenase/oxidase, C-terminal
Occurring in:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial
IPR036250
Domain description: Acyl-CoA dehydrogenase-like, C-terminal
Occurring in:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial
IPR006091
Domain description: Acyl-CoA oxidase/dehydrogenase, middle domain
Occurring in:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial
IPR046373
Domain description: Acyl-CoA oxidase/dehydrogenase, middle domain superfamily
Occurring in:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial
IPR013786
Domain description: Acyl-CoA dehydrogenase/oxidase, N-terminal
Occurring in:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial
IPR009100
Domain description: Acyl-CoA dehydrogenase/oxidase, N-terminal and middle domain superfamily
Occurring in:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial
IPR034183
Domain description: Isovaleryl-CoA dehydrogenase
Occurring in:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial
IPR037069
Domain description: Acyl-CoA dehydrogenase/oxidase, N-terminal domain superfamily
Occurring in:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial

Structure domains

CATH CATH domains
1.20.140.10
Class: Mainly Alpha
Architecture: Up-down Bundle
Topology: Butyryl-CoA Dehydrogenase, subunit A; domain 3
Homology: Butyryl-CoA Dehydrogenase, subunit A, domain 3
Occurring in:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial
The deposited structure of PDB entry 1ivh contains 4 copies of CATH domain 1.20.140.10 (Butyryl-CoA Dehydrogenase, subunit A; domain 3) in Isovaleryl-CoA dehydrogenase, mitochondrial. Showing 1 copy in chain A.
2.40.110.10
Class: Mainly Beta
Architecture: Beta Barrel
Topology: Butyryl-CoA Dehydrogenase, subunit A; domain 2
Homology: Butyryl-CoA Dehydrogenase, subunit A, domain 2
Occurring in:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial
The deposited structure of PDB entry 1ivh contains 4 copies of CATH domain 2.40.110.10 (Butyryl-CoA Dehydrogenase, subunit A; domain 2) in Isovaleryl-CoA dehydrogenase, mitochondrial. Showing 1 copy in chain A.
1.10.540.10
Class: Mainly Alpha
Architecture: Orthogonal Bundle
Topology: Butyryl-Coa Dehydrogenase, subunit A; domain 1
Homology: Acyl-CoA dehydrogenase/oxidase, N-terminal domain
Occurring in:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial
The deposited structure of PDB entry 1ivh contains 4 copies of CATH domain 1.10.540.10 (Butyryl-Coa Dehydrogenase, subunit A; domain 1) in Isovaleryl-CoA dehydrogenase, mitochondrial. Showing 1 copy in chain A.
SCOP SCOP annotations
47204
Class: All alpha proteins
Fold: Bromodomain-like
Superfamily: Acyl-CoA dehydrogenase C-terminal domain-like
Occurring in:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial
The deposited structure of PDB entry 1ivh contains 4 copies of SCOP domain 47204 (Medium chain acyl-CoA dehydrogenase-like, C-terminal domain) in Isovaleryl-CoA dehydrogenase, mitochondrial. Showing 1 copy in chain A.
56646
Class: Multi-domain proteins (alpha and beta)
Fold: Acyl-CoA dehydrogenase NM domain-like
Superfamily: Acyl-CoA dehydrogenase NM domain-like
Occurring in:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial
The deposited structure of PDB entry 1ivh contains 4 copies of SCOP domain 56646 (Medium chain acyl-CoA dehydrogenase, NM (N-terminal and middle) domains) in Isovaleryl-CoA dehydrogenase, mitochondrial. Showing 1 copy in chain A.

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