Rabbit anti-Human ACADSB Polyclonal Antibody

Uniprot Id

P45954

Target Species

Human

Target Name

ACADSB

Target Full Name

Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial

Target Function

Short and branched chain specific acyl-CoA dehydrogenase that catalyzes the removal of one hydrogen from C-2 and C-3 of the fatty acyl-CoA thioester, resulting in the formation of trans-2-enoyl-CoA. Among the different mitochondrial acyl-CoA dehydrogenases, acts specifically on short and branched chain acyl-CoA derivatives such as (S)-2-methylbutyryl-CoA as well as short straight chain acyl-CoAs such as butyryl-CoA. Plays an important role in the metabolism of L-isoleucine by catalyzing the dehydrogenation of 2-methylbutyryl-CoA, one of the steps of the L-isoleucine catabolic pathway. Can also act on valproyl-CoA, a metabolite of valproic acid, an antiepileptic drug.

Target Involvement

Short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD)

Target Subcellular Location

Mitochondrion matrix.

Target Protein Families

Acyl-CoA dehydrogenase family

Target Tissue Specificity

Ubiquitously expressed.

Target Synonyms

2 MEBCAD; 2 methyl branched chain acyl CoA dehydrogenase; 2 methylbutyryl CoA dehydrogenase; 2 methylbutyryl coenzyme A dehydrogenase; 2-MEBCAD; 2-methyl branched chain acyl-CoA dehydrogenase; 2-methylbutyryl-CoA dehydrogenase; 2-methylbutyryl-coenzyme A dehydrogenase; ACAD7; ACADSB; ACDSB_HUMAN; acyl CoA dehydrogenase; short/branched chain; acyl Coenzyme A dehydrogenase short branched chain; mitochondrial; OTTHUMP00000020685; OTTHUMP00000046795; SBCAD; Short/branched chain specific acyl-CoA dehydrogenase; short/branched chain specific acyl-CoA dehydrogenase; mitochondrial

Target Background

Short/branched chain acyl-CoA dehydrogenase(ACADSB) is 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. Substrate specificity is the primary characteristic used to define members of this gene family. The ACADSB gene product has the greatest activity towards the short branched chain acyl-CoA derivative, (S)-2-methylbutyryl-CoA, but also reacts significantly with other 2-methyl branched chain substrates and with short straight chain acyl-CoAs. The cDNA encodes for a mitochondrial precursor protein which is cleaved upon mitochondrial import and predicted to yield a mature peptide of approximately 43.7-KDa.