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bifunctional peroxisomal enzyme [includes: enoyl-CoA hydratase/3,2-trans-enoyl-CoA isomerase; 3-hydroxyacyl-CoA dehydrogenase] (PBE, PBFE, EHHADH, ECHD)
Function:
- fatty acid beta-oxidation cycle; step 2
- fatty acid beta-oxidation cycle; step 3
- fatty acid beta-oxidation cycle; auxiliary isomerization steps
(3S)-3-hydroxyacyl-CoA trans-2(or 3)-enoyl-CoA + H2O
(S)-3-hydroxyacyl-CoA + NAD+ 3-oxoacyl-CoA + NADH
3-cis-dodecenoyl-CoA 2-trans-dodecenoyl-CoA
Structure:
- monomer
- in the N-terminal section, belongs to the enoyl-CoA hydratase/isomerase family
- in the C-terminal section; belongs to the 3-hydroxyacyl- CoA dehydrogenase family
Compartment: peroxisome
Expression:
- liver & kidney
- lower amounts seen in the brain
Pathology:
- absent in patients suffering with peroxisomal disorders including as Zellweger syndrome, neonatal adrenoleukodystrophy & infantile Refsum disease
General
2,4-dienoyl CoA reductase
3-OH acyl CoA dehydrogenase
3,2-trans enoyl CoA isomerase; dodecenoyl CoA delta-isomerase (DCI, ECI1)
Properties
SIZE: entity length = 723 aa
MW = 79 kD
COMPARTMENT: peroxisome
MOTIF: Enoyl-CoA hydratase / isomerase {2-282}
MOTIF: glutamate residue {E104}
glutamate residue {E124}
3-hydroxyacyl-CoA dehydrogenase {283-572}
MOTIF: Ser phosphorylation site {S359}
microbody-targeting signal {721-723}
Database Correlations
OMIM correlations
UniProt Q08426
PFAM correlations
KEGG correlations
ENZYME correlations
References
UniProt :accession Q08426