P16219.1
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
NCBI Official Full Name
Short-chain specific acyl-CoA dehydrogenase, mitochondrial
NCBI Official Synonym Full Names
acyl-CoA dehydrogenase, C-2 to C-3 short chain
NCBI Official Synonym Symbols
NCBI Protein Information
short-chain specific acyl-CoA dehydrogenase, mitochondrial; butyryl-CoA dehydrogenase; unsaturated acyl-CoA reductase; acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain; mitochondrial short-chain specific acyl-CoA dehydrogenase
UniProt Protein Name
Short-chain specific acyl-CoA dehydrogenase, mitochondrial
UniProt Synonym Protein Names
Butyryl-CoA dehydrogenase
UniProt Synonym Gene Names
UniProt Entry Name
ACADS_HUMAN
NCBI Summary for ACADS
This gene encodes a tetrameric mitochondrial flavoprotein, which is a member of the acyl-CoA dehydrogenase family. This enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Mutations in this gene have been associated with short-chain acyl-CoA dehydrogenase (SCAD) deficiency. Alternative splicing results in two variants which encode different isoforms. [provided by RefSeq, Oct 2014]
UniProt Comments for ACADS
ACADS: Defects in ACADS are the cause of acyl-CoA dehydrogenase short-chain deficiency (ACADSD). It is an autosomal recessive disorder resulting in acute acidosis and muscle weakness in infants, and a form of lipid-storage myopathy in adults. Belongs to the acyl-CoA dehydrogenase family.
Protein type: Lipid Metabolism - fatty acid; Mitochondrial; Carbohydrate Metabolism - butanoate; Oxidoreductase; EC 1.3.8.1; Amino Acid Metabolism - valine, leucine and isoleucine degradation
Chromosomal Location of Human Ortholog: 12q24.31
Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial membrane; nucleus
Molecular Function: acyl-CoA dehydrogenase activity; butyryl-CoA dehydrogenase activity; FAD binding; acyl-CoA binding
Biological Process: response to starvation; fatty acid beta-oxidation; response to glucocorticoid stimulus; cellular lipid metabolic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; butyrate catabolic process; protein homotetramerization
Disease: Acyl-coa Dehydrogenase, Short-chain, Deficiency Of
Research Articles on ACADS
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Products associated with anti-ACADS antibody
Pathways associated with anti-ACADS antibody
Diseases associated with anti-ACADS antibody
Organs/Tissues associated with anti-ACADS antibody
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