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The effect of a Glu370Asp mutation in Glutaryl-CoA Dehydrogenase on Proton Transfer to the Dienolate Intermediate

Source organism: Homo sapiens
Biochemical function: flavin adenine dinucleotide binding
Biological process: fatty-acyl-CoA biosynthetic process
Cellular component: mitochondrial matrix

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EC 1.3.8.6: Glutaryl-CoA dehydrogenase (ETF)

Reaction catalysed:
(1a) glutaryl-CoA + electron-transfer flavoprotein = (E)-glutaconyl-CoA + reduced electron-transfer flavoprotein
Systematic name:
Glutaryl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase (decarboxylating)
Alternative Name(s):
  • Glutaryl coenzyme A dehydrogenase
  • Glutaryl-CoA dehydrogenase
  • Glutaryl-CoA:(acceptor) 2,3-oxidoreductase (decarboxylating)

GO terms

Sequence families

Pfam Protein families (Pfam)
PF00441
Domain description: Acyl-CoA dehydrogenase, C-terminal domain
Occurring in:
  1. Glutaryl-CoA dehydrogenase, mitochondrial
The deposited structure of PDB entry 2r0n contains 1 copy of Pfam domain PF00441 (Acyl-CoA dehydrogenase, C-terminal domain) in Glutaryl-CoA dehydrogenase, mitochondrial. Showing 1 copy in chain A.

PF02770
Domain description: Acyl-CoA dehydrogenase, middle domain
Occurring in:
  1. Glutaryl-CoA dehydrogenase, mitochondrial
The deposited structure of PDB entry 2r0n contains 1 copy of Pfam domain PF02770 (Acyl-CoA dehydrogenase, middle domain) in Glutaryl-CoA dehydrogenase, mitochondrial. Showing 1 copy in chain A.

PF02771
Domain description: Acyl-CoA dehydrogenase, N-terminal domain
Occurring in:
  1. Glutaryl-CoA dehydrogenase, mitochondrial
The deposited structure of PDB entry 2r0n contains 1 copy of Pfam domain PF02771 (Acyl-CoA dehydrogenase, N-terminal domain) in Glutaryl-CoA dehydrogenase, mitochondrial. Showing 1 copy in chain A.

InterPro InterPro annotations
IPR006089
Domain description: Acyl-CoA dehydrogenase, conserved site
Occurring in:
  1. Glutaryl-CoA dehydrogenase, mitochondrial
IPR009075
Domain description: Acyl-CoA dehydrogenase/oxidase, C-terminal
Occurring in:
  1. Glutaryl-CoA dehydrogenase, mitochondrial
IPR036250
Domain description: Acyl-CoA dehydrogenase-like, C-terminal
Occurring in:
  1. Glutaryl-CoA dehydrogenase, mitochondrial
IPR006091
Domain description: Acyl-CoA oxidase/dehydrogenase, middle domain
Occurring in:
  1. Glutaryl-CoA dehydrogenase, mitochondrial
IPR046373
Domain description: Acyl-CoA oxidase/dehydrogenase, middle domain superfamily
Occurring in:
  1. Glutaryl-CoA dehydrogenase, mitochondrial
IPR013786
Domain description: Acyl-CoA dehydrogenase/oxidase, N-terminal
Occurring in:
  1. Glutaryl-CoA dehydrogenase, mitochondrial
IPR009100
Domain description: Acyl-CoA dehydrogenase/oxidase, N-terminal and middle domain superfamily
Occurring in:
  1. Glutaryl-CoA dehydrogenase, mitochondrial
IPR037069
Domain description: Acyl-CoA dehydrogenase/oxidase, N-terminal domain superfamily
Occurring in:
  1. Glutaryl-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. Glutaryl-CoA dehydrogenase, mitochondrial
The deposited structure of PDB entry 2r0n contains 1 copy of CATH domain 1.20.140.10 (Butyryl-CoA Dehydrogenase, subunit A; domain 3) in Glutaryl-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. Glutaryl-CoA dehydrogenase, mitochondrial
The deposited structure of PDB entry 2r0n contains 1 copy of CATH domain 2.40.110.10 (Butyryl-CoA Dehydrogenase, subunit A; domain 2) in Glutaryl-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. Glutaryl-CoA dehydrogenase, mitochondrial
The deposited structure of PDB entry 2r0n contains 1 copy of CATH domain 1.10.540.10 (Butyryl-Coa Dehydrogenase, subunit A; domain 1) in Glutaryl-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. Glutaryl-CoA dehydrogenase, mitochondrial
The deposited structure of PDB entry 2r0n contains 1 copy of SCOP domain 47204 (Medium chain acyl-CoA dehydrogenase-like, C-terminal domain) in Glutaryl-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. Glutaryl-CoA dehydrogenase, mitochondrial
The deposited structure of PDB entry 2r0n contains 1 copy of SCOP domain 56646 (Medium chain acyl-CoA dehydrogenase, NM (N-terminal and middle) domains) in Glutaryl-CoA dehydrogenase, mitochondrial. Showing 1 copy in chain A.

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