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This reaction is essential for the subsequent steps in beta oxidation that lead to the production of acetyl-CoA, NADH, and FADH2, which are important for generating ATP, the energy currency of the cell. Long-chain hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency is a condition that affects mitochondrial function due to enzyme impairments.
Following glycolysis, the citric acid cycle is activated by the production of acetyl-CoA. The oxidation of pyruvate by pyruvate dehydrogenase in the matrix produces CO 2, acetyl-CoA, and NADH. Beta oxidation of fatty acids serves as an alternate catabolic pathway that produces acetyl-CoA, NADH, and FADH 2. [1]
This beta oxidation reaction is repeated until the fatty acid has been completely reduced to acetyl-CoA or, in the case of fatty acids with odd numbers of carbon atoms, acetyl-CoA and 1 molecule of propionyl-CoA per molecule of fatty acid. Each beta oxidative cut of the acyl-CoA molecule eventually yields 5 ATP molecules in oxidative ...
Figure 1: Reaction Mechanism of Enoyl-CoA Isomerase. Enoyl-CoA isomerase is involved in the beta-oxidation, one of the most frequently used pathways in fatty acid degradation, of unsaturated fatty acids with double bonds at odd-numbered carbon positions. [2]
The following reaction is the oxidation of the fatty acid by FAD to afford an α,β-unsaturated fatty acid thioester of coenzyme A: ACADs can be categorized into three distinct groups based on their specificity for short-, medium-, or long-chain fatty acid acyl-CoA substrates.
Thiolases are a family of evolutionarily related enzymes.Two different types of thiolase [4] [5] [6] are found both in eukaryotes and in prokaryotes: acetoacetyl-CoA thiolase (EC 2.3.1.9) and 3-ketoacyl-CoA thiolase (EC 2.3.1.16). 3-ketoacyl-CoA thiolase (also called thiolase I) has a broad chain-length specificity for its substrates and is involved in degradative pathways such as fatty acid ...
Example of Beta Oxidation using Stearic Acid. Beta oxidation, as well as alpha-oxidation, also occurs in the peroxisome. [1] The peroxisome handles beta oxidation of fatty acids that have more than 20 carbons in their chain because the peroxisome contains very-long-chain Acyl-CoA synthetases. [9] These enzymes are better equipped to oxidize ...
Enoyl-CoA hydratase (ECH) or crotonase [1] is an enzyme EC 4.2.1.17 that hydrates the double bond between the second and third carbons on 2-trans/cis-enoyl-CoA: [2]. ECH is essential to metabolizing fatty acids in beta oxidation to produce both acetyl CoA and energy in the form of ATP.