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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. Note: This description may include information from UniProtKB.
Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC; Lipid Metabolism - fatty acid; Mitochondrial; Oxidoreductase
Chromosomal Location of Human Ortholog: 10q26.13
Cellular Component: extracellular exosome; mitochondrial matrix; mitochondrion
Molecular Function: acyl-CoA dehydrogenase activity; FAD binding
Biological Process: branched-chain amino acid catabolic process; fatty acid metabolic process
Disease: 2-methylbutyryl-coa Dehydrogenase Deficiency
Reference #:  P45954 (UniProtKB)
Alt. Names/Synonyms: 2-MEBCAD; 2-methyl branched chain acyl-CoA dehydrogenase; 2-methylbutyryl-CoA dehydrogenase; 2-methylbutyryl-coenzyme A dehydrogenase; ACAD7; ACADSB; ACDSB; acyl-Coenzyme A dehydrogenase, short/branched chain; SBCAD; Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
Gene Symbols: ACADSB
Molecular weight: 47,485 Da
Basal Isoelectric point: 6.53  Predict pI for various phosphorylation states
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Protein Structure Not Found.
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