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Acyl CoA dehydrogenase

German: Acyl-CoA-Dehydrogenase

1 Definition

Acyl CoA dehydrogenases are mitochondrial enzymes that catalyze the oxidative degradation of fatty acids which is the first step of beta oxidation.

2 Details

The basis of beta oxidation and its enzymes is to degrade fatty acids in a cyclical metabolic pathway in the mitochondrial matrix mainly through oxidation, i.e. by taking away their electrons. Those electrons are subsequently used in the respiratory chain to build up the mitochondrial proton gradient.

3 Biochemistry

3.1 Enzyme

As in the citrate cycle, dehydrogenases are essential enzymes for the degradation of fatty acids. The acyl CoA dehydrogenase enzyme catalyzes an FAD-dependent oxidation (=dehydration) of -CH2-CH2 groups.

3.2 Reaction

The first step of beta oxidation is to create a double bond between the α- and β-C-atoms. The substrate is acyl CoA, the acyl group of which initially only exhibits a chain of -CH2-CH2 units. To prepare for the elimination of the future acetyl-CoA, a double bond is added to the site of elimination. The electrons resulting from the oxidation are attached to the enzyme-bound FAD. The resulting FADH2 is then transported to the respiratory chain. The functional groups adjacent to the double bond point in opposite directions, i.e. they assume a trans configuration. The product of the reaction contains a -HC=CH- group with an adjacent carbonyl group, i.e. an enoyl group, which is why it is referred to as trans-enoyl-CoA.

4 Clinical significance

Hereditary defects of acyl CoA dehydrogenase lead to disruptions in the degradation of fatty acids.

see also: acyl CoA dehydrogenase deficiency

5 Literature

Joachim Rassow et al.: Duale Reihe Biochemie, Thieme, 2. edition

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