peroxisomal multifunctional enzyme type 2; MFE-2; 17-beta-hydroxysteroid dehydrogenase 4; 17-beta-HSD 4; D-bifunctional protein; DBP; multifunctional protein 2; MPF-2; contains: (3R)-hydroxyacyl-CoA dehydrogenase; enoyl-CoA hydratase 2; 3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholest-24-enoyl-CoA hydratase (HSD17B4, EDH17B4)

Function

• bifunctional enzyme acting on peroxisomal beta-oxidation pathway for fatty acids
• catalyzes formation of 3-ketoacyl-CoA intermediates from both straight-chain & 2-methyl-branched-chain fatty acids
• fatty acid beta-oxidation cycle, steps 2 & 3

Kinetic parameters:

• KM=10 uM for D-3-hydroxy-octanoyl-CoA
• KM=13 uM for NAD
• KM=2.7 uM for 3-ketooctanoyl-CoA
• KM=5.4 uM for NADH
• Vmax=8.8 umol/min/mg enzyme

Structure

• homodimer
• belongs to the short-chain dehydrogenases/reductases (SDR) family
• contains 1 MaoC-like domain
• contains 1 SCP2 domain

Compartment

peroxisome

Expression

highest in liver, heart, prostate, testis

Pathology

• defects in HSD17B4 are a cause of D-bifunctional protein deficiency
• defects in HSD17B4 are the cause of Perrault syndrome

Laboratory

• HSD17B4 gene mutation

Notes

• HSD17B4 is found both as a full length peptide & in a cleaved peptide

More general terms

• multifunctional enzyme
• acyl CoA dehydrogenase
• 3-OH acyl CoA dehydrogenase
• hydro-lyase, hydratase or dehydratase
• phosphoprotein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=3295
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:3295
• OMIM: https://mirror.omim.org/entry/233400
• OMIM: https://mirror.omim.org/entry/261515
• OMIM: https://mirror.omim.org/entry/601860
• UniProt: http://www.uniprot.org/uniprot/P51659.html