Enoyl-CoA hydratase

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spinqualitylabelsall models Structure of enoyl-CoA hydratase/isomerase complex with acetoacetyl-CoA and sulfate (PDB code 4fn8). Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image Contents 1 Function 2 Disease 3 Relevance 4 Structural highlights Function Enoyl-CoA hydratase (ECH) is part of the fatty acid metabolism. ECH hydrates the double bond between the second and third carbons in acyl-CoA. ECH1 or short-chain ECH is mitochondrial. ECH1 processes enoyl-CoA thioesters from C4 to C16[1]. Disease ECH1 mutations cause combined respiratory chain deficiency resulting inn Leigh syndrome[2]. Relevance Structural highlights

3D structures of enoyl-CoA hydratase

Updated on 19-January-2016

References

1. ↑ Agnihotri G, Liu HW. Enoyl-CoA hydratase. reaction, mechanism, and inhibition. Bioorg Med Chem. 2003 Jan 2;11(1):9-20. PMID:12467702
2. ↑ Sakai C, Yamaguchi S, Sasaki M, Miyamoto Y, Matsushima Y, Goto Y. ECHS1 mutations cause combined respiratory chain deficiency resulting in Leigh syndrome. Hum Mutat. 2015 Feb;36(2):232-9. doi: 10.1002/humu.22730. PMID:25393721 doi:http://dx.doi.org/10.1002/humu.22730