Enoyl-CoA hydratase

From Proteopedia

proteopedia linkproteopedia link

spinqualitylabelsall models Structure of enoyl-CoA hydratase complex with hexadienoyl-CoA (PDB code 1mj3). Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image Contents 1 Function 2 Disease 3 Structural highlights 4 3D structures of enoyl-CoA hydratase 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]. Structural highlights ; . ECH . [3] 3D structures of enoyl-CoA hydratase Enoyl-CoA hydratase 3D structures

3D structures of enoyl-CoA hydratase

Updated on 20-June-2019

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
3. ↑ Bell AF, Feng Y, Hofstein HA, Parikh S, Wu J, Rudolph MJ, Kisker C, Whitty A, Tonge PJ. Stereoselectivity of enoyl-CoA hydratase results from preferential activation of one of two bound substrate conformers. Chem Biol. 2002 Nov;9(11):1247-55. PMID:12445775