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Fig. 1 | Orphanet Journal of Rare Diseases

Fig. 1

From: Genetic pathogenesis, diagnosis, and treatment of short-chain 3-hydroxyacyl-coenzyme A dehydrogenase hyperinsulinism

Fig. 1

SCHAD is involved in fatty acidsβ-oxidation: Acyl-CoAs are degraded in mitochondria by four enzymatic steps of β-oxidation. The process of each cycle: the first step is dehydrogenation by FAD, the acyl-CoA dehydrogenase catalyzed acyl-CoA into trans-Δ2-enoyl-CoA. Then a hydration catalyzed by an enoyl-CoA hydratase happened, the product is 3-hydroxyacyl-CoA. During the third step, 3-hydroxyacyl-CoA is dehydrogenated into 3-ketoacyl-CoA by 3-hydroxyacyl-CoA dehydrogenase. In the end, thiolase cleaves 3-keto-CoA into a two-carbon chain shortened acyl-CoA and an acetyl-CoA. The process continues until all the carbon in the fatty acid has been converted to acetyl-CoA. SCHAD short-chain L-3-hydroxyacyl-CoA dehydrogenase; ETFDH electron transfer flavoprotein-ubiquinone oxidoreductase; OXPHOS oxidative phosphorylation system

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