What does enoyl-CoA hydratase do?

Enoyl-CoA hydratase (ECH) catalyzes the second step in the physiologically important beta-oxidation pathway of fatty acid metabolism. This enzyme facilitates the syn-addition of a water molecule across the double bond of a trans-2-enoyl-CoA thioester, resulting in the formation of a beta-hydroxyacyl-CoA thioester.

Is Enoyl-CoA Hydratase a lyase?

The systematic name of this enzyme class is long-chain-(3S)-3-hydroxyacyl-CoA hydro-lyase. This enzyme is also called long-chain enoyl coenzyme A hydratase.

What is the coenzyme that functions with 2 4 Dienoyl CoA reductase?

DECR1 participates in the beta oxidation and metabolism of polyunsaturated fatty enoyl-CoA esters. Specifically, it catalyzes the reduction of 2,4 dienoyl-CoA thioesters of varying length by NADPH cofactor to 3-trans-enoyl-CoA of equivalent length.

What is the function of enoyl-CoA hydratase?

Enoyl-CoA hydratase (ECH) catalyzes the second step in the physiologically important beta-oxidation pathway of fatty acid metabolism. This enzyme facilitates the syn -addition of a water molecule across the double bond of a trans -2-enoyl-CoA thioester, resulting in the formation of a β-hydroxyacyl-CoA thioester.

What causes short chain enoyl-CoA hydratase deficiency?

Abstract Background: Short-chain enoyl-CoA hydratase (SCEH or ECHS1) deficiency is a rare congenital metabolic disorder caused by biallelic mutations in the ECHS gene.

Which is short chain enoyl-CoA hydratase ( SCEH ) gene?

The ECHS1 gene encodes short-chain enoyl-CoA hydratase (SCEH; EC 4.2.1.17 ), which catalyzes the second step in mitochondrial fatty acid beta-oxidation (summary by Kanazawa et al., 1993 ). The enzyme is also active in the isoleucine and valine catabolic pathways.

What happens if enoyl CoA gives off more than one product?

If the enzyme is unclear, react it with an enoyl CoA Derivative. If the enzyme gives off more than one product, it is a multifunctional enzyme. If it gives off one product, it is solely enoyl Co-Al isomerase. Mitochondria (both short- and long-chain) of rat liver contain more than one enoyl Co-A isomerase.