ACAD8
ACAD8 (acyl-CoA dehydrogenase family member 8) encodes isobutyryl-CoA dehydrogenase, a mitochondrial flavoprotein enzyme of the acyl-CoA dehydrogenase family. It acts in the catabolism of the branched-chain amino acid valine. Browse Assay Genie's current range of ACAD8 research reagents below, or read on for the biology behind the target.
ACAD8 is an isobutyryl-CoA dehydrogenase. It catalyses the conversion of 2-methylpropanoyl-CoA to (2E)-2-methylpropenoyl-CoA in the valine catabolic pathway. To a lesser extent, the enzyme can also oxidise (2S)-2-methylbutanoyl-CoA. As a member of the acyl-CoA dehydrogenase family, it carries out dehydrogenation of acyl-CoA derivatives, a reaction type that features in the metabolism of both fatty acids and branched-chain amino acids.
The ACAD8 gene encodes a mitochondrial protein, and UniProt records its subcellular location as the mitochondrion. HGNC places the gene in the acyl-CoA dehydrogenase family and in the wider group of flavoproteins. The enzyme carries the EC number 1.3.8.5. The mouse ortholog is designated Acad8, which allows comparative work on the ACAD8 protein in rodent models. Researchers studying the ACAD8 gene often examine it alongside other family members that handle different acyl-CoA substrates, such as ACADM and ACADS.
Defects in ACAD8 are the cause of isobutyryl-CoA dehydrogenase deficiency, which links the enzyme directly to inherited disorders of branched-chain amino acid metabolism. HGNC lists earlier names for the gene as acyl-Coenzyme A dehydrogenase family, member 8, and acyl-CoA dehydrogenase family, member 8. ACAD8 is therefore a useful reference point for work on valine catabolism, mitochondrial acyl-CoA dehydrogenases and the biochemical basis of this deficiency.
ACAD8 catalyses a step in the valine catabolic pathway, converting 2-methylpropanoyl-CoA to (2E)-2-methylpropenoyl-CoA.
Defects in ACAD8 cause isobutyryl-CoA dehydrogenase deficiency, making it a focus for studies of branched-chain amino acid disorders.
As a mitochondrial flavoprotein of the acyl-CoA dehydrogenase family, ACAD8 is a model for dehydrogenation of branched-chain acyl-CoA substrates.
ACAD8 also acts, to a lesser extent, on (2S)-2-methylbutanoyl-CoA, which supports comparison of substrate preference across acyl-CoA dehydrogenases.
ACAD8 is a mitochondrial isobutyryl-CoA dehydrogenase. It converts 2-methylpropanoyl-CoA to (2E)-2-methylpropenoyl-CoA in the valine catabolic pathway and, to a lesser extent, oxidises (2S)-2-methylbutanoyl-CoA. It belongs to the acyl-CoA dehydrogenase family.
The UniProt protein name is isobutyryl-CoA dehydrogenase, mitochondrial. The HGNC full name is acyl-CoA dehydrogenase family member 8, and earlier names include acyl-Coenzyme A dehydrogenase family, member 8. No alternative gene symbols are recorded.
Defects in ACAD8 cause isobutyryl-CoA dehydrogenase deficiency. The mouse ortholog is Acad8, so rodent work is possible; check the datasheet of any product for its validated reactivity.
Other members of the acyl-CoA dehydrogenase family involved in fatty acid and branched-chain amino acid metabolism.
Gene and protein annotations from HGNC, NCBI Gene and UniProt Q9UKU7; product specifications from Assay Genie datasheets.