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isobutyryl-CoA dehydrogenase, mitochondrial; activator-recruited cofactor 42 kD component; ARC42; acyl-CoA dehydrogenase family member 8; ACAD-8 (ACAD8, ARC42, IBD)
Function:
- isobutyryl-CoA dehydrogenase
- very high activity toward isobutyryl-CoA
- functions in valine catabolism
- component of the ARC/DRIP complex
- within the ARC/DRIP complex, ACAD8 plays a role in transcriptional coactivation
- amino-acid degradation; L-valine degradation
isobutyryl-CoA + ETF = methylacrylyl-CoA + reduced ETF
Cofactor: FAD
Structure:
- homotetramer, formed by a dimer of dimers
- belongs to the acyl-CoA dehydrogenase family
Compartment: mitochondria
Expression:
- expressed in heart, lung, brain, skeletal muscle, pancreas & placenta
- weakly expressed in liver & kidney
Pathology:
- defects in ACAD8 are the cause of isobutyryl-CoA dehydrogenase deficiency
General
dehydrogenase
mitochondrial protein
transcription factor (TF)
Properties
SIZE: entity length = 415 aa
MW = 45 kD
COMPARTMENT: mitochondria
MOTIF: cofactor-binding site [158-167]
COFACTOR-BOUND: flavin adenine dinucleotide
MOTIF: binding site
SITE: 167-167
FOR-BINDING-OF: Substrate
cofactor-binding site [191-193]
COFACTOR-BOUND: flavin adenine dinucleotide
Substrate binding {274-277}
cofactor-binding site [302-302]
FOR-BINDING-OF: flavin adenine dinucleotide
cofactor-binding site [312-313]
COFACTOR-BOUND: flavin adenine dinucleotide
cofactor-binding site [371-375]
COFACTOR-BOUND: flavin adenine dinucleotide
glutamate residue {E398}
binding site
SITE: 399-399
FOR-BINDING-OF: Substrate
cofactor-binding site [400-402]
COFACTOR-BOUND: flavin adenine dinucleotide
binding site
SITE: 410-410
FOR-BINDING-OF: Substrate
Database Correlations
OMIM correlations
MORBIDMAP 604773
UniProt Q9UKU7
PFAM correlations
Entrez Gene 27034
Kegg hsa:27034
ENZYME 1.3.99._
References
- UniProt :accession Q9UKU7
- GeneReviews
http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/ACAD8
Component-of
activator-recruited cofactor (ARC)/DRIP