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

More general terms

• dehydrogenase
• mitochondrial protein
• transcription factor (TF)

Component of

• activator-recruited cofactor (ARC)/DRIP

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=27034
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:27034
• OMIM: https://mirror.omim.org/entry/604773
• OMIM: https://mirror.omim.org/entry/611283
• UniProt: http://www.uniprot.org/uniprot/Q9UKU7.html