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. Belongs to the acyl-CoA dehydrogenase family. Ubiquitous. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
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Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 1.3.8.5; Lipid Metabolism - fatty acid; Mitochondrial; Oxidoreductase |
Chromosomal Location of human Ortholog: 10q26.13 |
Cellular Component:
mitochondrial matrix; mitochondrion
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Molecular Function:
2-methylacyl-CoA dehydrogenase activity; acyl-CoA dehydrogenase activity; butyryl-CoA dehydrogenase activity; flavin adenine dinucleotide binding; identical protein binding; isobutyryl-CoA:FAD oxidoreductase activity; short-chain-acyl-CoA dehydrogenase activity
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Biological Process:
fatty acid metabolic process; isoleucine catabolic process
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Disease: 2-methylbutyryl-coa Dehydrogenase Deficiency
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Reference #:
P45954
(UniProtKB)
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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-CoA dehydrogenase short/branched chain; acyl-Coenzyme A dehydrogenase, short/branched chain; SBCAD; Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
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Gene Symbols: ACADSB
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Molecular weight:
47,485 Da
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Basal Isoelectric point:
6.53
Predict pI for various phosphorylation states
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