Proteins > Short-chain specific acyl-CoA dehydrogenase, mitochondrial (SCAD)
Short-chain specific acyl-CoA dehydrogenase,
mitochondrial (SCAD)
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UniProt Number:
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P16219
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Alternate Names:
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Butyryl-CoA dehydrogenase, ACADS
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Structure and Function:
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Acyl-CoA dehydrogenases are a class of enzymes that catalyze the initial step in each cycle of fatty acid β-oxidation in the mitochondria of cells. Their action results in the introduction of a trans double-bond between C2 and C3 of the acyl-CoA thioester substrate. Acyl-CoA dehydrogenases can be categorized into three distinct groups based on their specificity for short-, medium-, or long-chain fatty acid acyl-CoA substrates. Short chain specific acyl-CoA dehydrogenases target fatty acids of 4-6 C length.
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Disease Associations:
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Defects in ACADS are the cause of acyl-CoA dehydrogenase short-chain deficiency (ACADSD) [MIM:201470]. It is an autosomal recessive disorder resulting in acute acidosis and muscle weakness in infants, and a form of lipid-storage myopathy in adults.
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Monoclonal Antibodies
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