ID:ACAD8_HUMAN DESCRIPTION: RecName: Full=Isobutyryl-CoA dehydrogenase, mitochondrial; EC=1.3.99.-; AltName: Full=Activator-recruited cofactor 42 kDa component; Short=ARC42; AltName: Full=Acyl-CoA dehydrogenase family member 8; Short=ACAD-8; Flags: Precursor; FUNCTION: Has very high activity toward isobutyryl-CoA. Is an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Plays a role in transcriptional coactivation within the ARC complex. CATALYTIC ACTIVITY: Isobutyryl-CoA + ETF = methylacrylyl-CoA + reduced ETF. COFACTOR: FAD. PATHWAY: Amino-acid degradation; L-valine degradation. SUBUNIT: Homotetramer, formed by a dimer of dimers. Subunit of the large multiprotein complex ARC/DRIP. SUBCELLULAR LOCATION: Mitochondrion. TISSUE SPECIFICITY: Detected at comparable levels in all tissues examined (heart, lung, brain, skeletal muscle, pancreas and placenta). Weakly expressed in liver and kidney. DISEASE: Defects in ACAD8 are the cause of isobutyryl-CoA dehydrogenase deficiency (IBDD) [MIM:611283]. The symptoms of IBDD generally appear until late in infancy or in childhood and can include poor feeding and growth (failure to thrive), a weakened and enlarged heart (dilated cardiomyopathy), seizures, and low numbers of red blood cells (anemia). SIMILARITY: Belongs to the acyl-CoA dehydrogenase family. WEB RESOURCE: Name=GeneReviews; URL="http://www.ncbi.nlm.nih.gov/sites/GeneTests/lab/gene/ACAD8";
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on Q9UKU7
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
