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

More general terms

• 3-OH acyl CoA dehydrogenase
• 3,2-trans enoyl CoA isomerase
• 2,4-dienoyl CoA reductase

Database

• UniProt: http://www.uniprot.org/uniprot/Q08426.html
• OMIM: https://mirror.omim.org/entry/261515
• OMIM: https://mirror.omim.org/entry/607037