Acyl Co A Dehydrogenase

Acyl Co A Dehydrogenase

Acyl-CoA dehydrogenases are a class of enzymes that function to 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. FAD is a required co-factor in the mechanism in order for the enzyme to bind to its appropriate substrate.

The following reaction is the oxidation of the fatty acid by FAD.

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. While different dehydrogenases target fatty acids of varying chain length, all types of acyl-CoA dehydrogenases are mechanistically similar. Differences in the enzyme occur based on the location of the active site along the amino acid sequence.

Acyl-CoA dehydrogenases are an important class of enzymes in mammalian cells because of their role in metabolizing fatty acids present in ingested food materials. This enzyme's action represents the first step in fatty acid metabolism (the process of breaking long chains of fatty acids into acetyl CoA molecules). Deficiencies in these enzymes are linked to genetic disorders involving fatty acid oxidation (i.e. metabolic disorders).

It is classified as EC 1.3.99.3.

Read more about Acyl Co A Dehydrogenase:  Structure, Mechanism, Deficiencies of Acyl CoA Dehydrogenase Linked To Metabolic Disease

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Acyl Co A Dehydrogenase - Deficiencies of Acyl CoA Dehydrogenase Linked To Metabolic Disease
... Deficiencies in acyl-CoA dehydrogenases result in decreased ability to oxidize fatty acids, thereby signifying metabolic dysfunction ... Medium-chain acyl-CoA dehydrogenase deficiencies MCADD are well known and characterized because they occur most commonly among acyl-CoA dehydrogenases, leading to fatty acid oxidation ... Some symptoms of medium-chain acyl-CoA dehydrogenase deficiency include intolerance to fasting, hypoglycemia, and sudden infant death syndrome ...