Structural Highlights
Length: 261 amino acids, Specific chains of 2-enoyl-coa hydratase are: a, b, c, d, e, f . 6 chain structure with sequence from Rattus norvegicus. ligand: CO8. The catalytic water is bound between Glu144 and Glu164 in the hydratase.
Function
Short-chain enoyl-CoA hydratase (ECHS1) is a multifunctional mitochondrial matrix enzyme that is involved in the oxidation of fatty acids and essential amino acids such as valine.
The active sites of the N- and the C-terminal parts catalyse,respectively, the second (2E-enoyl-CoA hydration) and third (3-hydroxy-acyl-CoA dehydrogenation) reactions
[2] of the b-oxidation pathway of fatty acid metabolism. Straight-chain enoyl-CoA thioesters from C4 up to at least C16 are processed, although with decreasing catalytic rate. The chains homologous to 2DUB: 1DUB, 1EY3.
(3S)-3-hydroxyacyl-CoA = trans-2(or 3)-enoyl-CoA + H(2)O
Disease
A catalytic water, bound
between the OE1-atoms of the two catalytic glutamates, Glu103 and
Glu123, plays an important role in the enoyl-CoA isomerase and the
enoyl-CoA hydratase reaction mechanism of MFE1.
Dysfunctional MFE2 is one of the most common reasons of peroxi- somal disorder in humans
Relevance
References
http://oca.weizmann.ac.il/oca-bin/ocashort?id=2DUB
http://jenalib.leibniz-fli.de/cgi-bin/3d_mapping.pl?CODE=2dub&MODE=biological1
https://www.uniprot.org/uniprot/P14604#function
