AKI70005.1
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
36,025 Da
NCBI Official Full Name
ACADSB, partial
NCBI Official Synonym Full Names
acyl-CoA dehydrogenase, short/branched chain
NCBI Protein Information
short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
UniProt Protein Name
Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
UniProt Synonym Protein Names
2-methyl branched chain acyl-CoA dehydrogenase; 2-MEBCAD; 2-methylbutyryl-coenzyme A dehydrogenase; 2-methylbutyryl-CoA dehydrogenase
UniProt Synonym Gene Names
UniProt Entry Name
ACDSB_HUMAN
NCBI Summary for ACADSB
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. [provided by RefSeq, Jul 2008]
UniProt Comments for ACADSB
ACADSB: Has greatest activity toward short branched chain acyl- CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl- CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent. Defects in ACADSB are the cause of short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD); also known as 2-methylbutyryl-CoA dehydrogenase deficiency or 2- methylbutyryl glycinuria. SBCADD is an autosomal recessive disorder and consists of a defect in catabolism of L-isoleucine which is characterized by an increase of 2-methylbutyrylglycine and 2-methylbutyrylcarnitine in blood and urine. Affected individuals have seizures and psychomotor delay as the main clinical features. Belongs to the acyl-CoA dehydrogenase family.
Protein type: Mitochondrial; EC 1.3.8.5; Oxidoreductase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Lipid Metabolism - fatty acid
Chromosomal Location of Human Ortholog: 10q26.13
Cellular Component: mitochondrion; mitochondrial matrix
Molecular Function: acyl-CoA dehydrogenase activity; FAD binding
Biological Process: branched chain family amino acid catabolic process; fatty acid metabolic process
Disease: 2-methylbutyryl-coa Dehydrogenase Deficiency
Research Articles on ACADSB
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Products associated with anti-ACADSB antibody
Pathways associated with anti-ACADSB antibody
Diseases associated with anti-ACADSB antibody
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