LMPD Database

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LMP001128

Record overview

LMPD IDLMP001128
Gene ID27034
SpeciesHomo sapiens (Human)
Gene Nameacyl-CoA dehydrogenase family, member 8
Gene SymbolACAD8
SynonymsACAD-8; ARC42
Alternate namesisobutyryl-CoA dehydrogenase, mitochondrial; activator-recruited cofactor 42 kDa component; acyl-Coenzyme A dehydrogenase family, member 8
Chromosome11
Map Location11q25
EC Number1.3.99.-
SummaryThis gene encodes a member of the acyl-CoA dehydrogenase family of enzymes that catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of fatty acids or branch chained amino acids. The encoded protein is a mitochondrial enzyme that functions in catabolism of the branched-chain amino acid valine. Defects in this gene are the cause of isobutyryl-CoA dehydrogenase deficiency.[provided by RefSeq, Nov 2009]
OrthologsView orthologs and multiple alignments for ACAD8

Proteins

isobutyryl-CoA dehydrogenase, mitochondrial
Refseq ID:NP_055199
Protein GI:7656849
UniProt ID:Q9UKU7
mRNA ID:NM_014384
Length:415
RefSeq Status:REVIEWED
MLWSGCRRFGARLGCLPGGLRVLVQTGHRSLTSCIDPSMGLNEEQKEFQKVAFDFAAREMAPNMAEWDQKELFPVDVMRKAAQLGFGGVYIQTDVGGSGL
SRLDTSVIFEALATGCTSTTAYISIHNMCAWMIDSFGNEEQRHKFCPPLCTMEKFASYCLTEPGSGSDAASLLTSAKKQGDHYILNGSKAFISGAGESDI
YVVMCRTGGPGPKGISCIVVEKGTPGLSFGKKEKKVGWNSQPTRAVIFEDCAVPVANRIGSEGQGFLIAVRGLNGGRINIASCSLGAAHASVILTRDHLN
VRKQFGEPLASNQYLQFTLADMATRLVAARLMVRNAAVALQEERKDAVALCSMAKLFATDECFAICNQALQMHGGYGYLKDYAVQQYVRDSRVHQILEGS
NEVMRILISRSLLQE