NP_080102.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Related Accession #
Molecular Weight
47,874 Da
NCBI Official Full Name
short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
NCBI Official Synonym Full Names
acyl-Coenzyme A dehydrogenase, short/branched chain
NCBI Official Synonym Symbols
SBCAD; 2-MEBCAD; BB066609; 1300003O09Rik [Similar Products]
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_MOUSE
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; Oxidoreductase; EC 1.3.8.5; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Lipid Metabolism - fatty acid
Cellular Component: mitochondrion
Molecular Function: short-branched-chain-acyl-CoA dehydrogenase activity; acyl-CoA dehydrogenase activity; electron carrier activity; FAD binding; oxidoreductase activity, acting on the CH-CH group of donors; oxidoreductase activity
Biological Process: acyl-CoA metabolic process; metabolic process; lipid metabolic process; fatty acid metabolic process
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Products associated with ACADSB peptide
Pathways associated with ACADSB peptide
Diseases associated with ACADSB peptide
Organs/Tissues associated with ACADSB peptide
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