Names & Taxonomy
- Uniprot ID:
- P51659
- Entry Name:
- DHB4_HUMAN
- Status:
- reviewed
- Protein Names:
- 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) (Short chain dehydrogenase/reductase family 8C member 1) [Cleaved into: (3R)-hydroxyacyl-CoA dehydrogenase (EC 1.1.1.n12); Enoyl-CoA hydratase 2 (EC 4.2.1.107) (EC 4.2.1.119) (3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholest-24-enoyl-CoA hydratase)]
- Gene Names:
- HSD17B4 EDH17B4 SDR8C1
- Gene Names Primary:
- HSD17B4
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 736
- Sequence:
- MGSPLRFDGRVVLVTGAGAGLGRAYALAFAERGALVVVNDLGGDFKGVGKGSLAADKVVEEIRRRGGKAVANYDSVEEGEKVVKTALDAFGRIDVVVNNAGILRDRSFARISDEDWDIIHRVHLRGSFQVTRAAWEHMKKQKYGRIIMTSSASGIYGNFGQANYSAAKLGLLGLANSLAIEGRKSNIHCNTIAPNAGSRMTQTVMPEDLVEALKPEYVAPLVLWLCHESCEENGGLFEVGAGWIGKLRWERTLGAIVRQKNHPMTPEAVKANWKKICDFENASKPQSIQESTGSIIEVLSKIDSEGGVSANHTSRATSTATSGFAGAIGQKLPPFSYAYTELEAIMYALGVGASIKDPKDLKFIYEGSSDFSCLPTFGVIIGQKSMMGGGLAEIPGLSINFAKVLHGEQYLELYKPLPRAGKLKCEAVVADVLDKGSGVVIIMDVYSYSEKELICHNQFSLFLVGSGGFGGKRTSDKVKVAVAIPNRPPDAVLTDTTSLNQAALYRLSGDWNPLHIDPNFASLAGFDKPILHGLCTFGFSARRVLQQFADNDVSRFKAIKARFAKPVYPGQTLQTEMWKEGNRIHFQTKVQETGDIVISNAYVDLAPTSGTSAKTPSEGGKLQSTFVFEEIGRRLKDIGPEVVKKVNAVFEWHITKGGNIGAKWTIDLKSGSGKVYQGPAKGAADTTIILSDEDFMEVVLGKLDPQKAFFSGRLKARGNIMLSQKLQMILKDYAKL
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Peroxisome.
Function
- Function:
- Bifunctional enzyme acting on the peroxisomal beta-oxidation pathway for fatty acids. Catalyzes the formation of 3-ketoacyl-CoA intermediates from both straight-chain and 2-methyl-branched-chain fatty acids.
- Pathway:
- Lipid metabolism; fatty acid beta-oxidation.
- Catalytic Activity:
- (R)-3-hydroxyacyl-CoA + NAD(+) = 3-oxoacyl-CoA + NADH.
- Kinetics:
- BIOPHYSICOCHEMICAL PROPERTIES: Kinetic parameters: KM=10 uM for D-3-hydroxy-octanoyl-CoA
- Active Site:
- ACT_SITE 164 164 Proton acceptor.
- Gene Ontology Go:
- intracellular membrane-bounded organelle
membrane
mitochondrion
peroxisomal matrix
peroxisomal membrane
peroxisome
17-beta-hydroxysteroid dehydrogenase (NAD+) activity
3-hydroxyacyl-CoA dehydrogenase activity
3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA hydratase activity
isomerase activity
long-chain-enoyl-CoA hydratase activity
protein homodimerization activity
receptor binding
alpha-linolenic acid metabolic process
androgen metabolic process
bile acid biosynthetic process
bile acid metabolic process
cellular lipid metabolic process
estrogen metabolic process
fatty acid beta-oxidation
fatty acid beta-oxidation using acyl-CoA oxidase
medium-chain fatty-acyl-CoA metabolic process
osteoblast differentiation
oxidation-reduction process
Sertoli cell development
small molecule metabolic process
unsaturated fatty acid metabolic process
very long-chain fatty acid metabolic process
very long-chain fatty-acyl-CoA metabolic process - Gene Ontology Biological Process:
- alpha-linolenic acid metabolic process
androgen metabolic process
bile acid biosynthetic process
bile acid metabolic process
cellular lipid metabolic process
estrogen metabolic process
fatty acid beta-oxidation
fatty acid beta-oxidation using acyl-CoA oxidase
medium-chain fatty-acyl-CoA metabolic process
osteoblast differentiation
oxidation-reduction process
Sertoli cell development
small molecule metabolic process
unsaturated fatty acid metabolic process
very long-chain fatty acid metabolic process
very long-chain fatty-acyl-CoA metabolic process - Gene Ontology Molecular Function:
- 17-beta-hydroxysteroid dehydrogenase (NAD+) activity
3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA hydratase activity
3-hydroxyacyl-CoA dehydrogenase activity
isomerase activity
long-chain-enoyl-CoA hydratase activity
protein homodimerization activity
receptor binding - Gene Ontology Cellular Component:
- intracellular membrane-bounded organelle
membrane
mitochondrion
peroxisomal matrix
peroxisomal membrane
peroxisome - Keywords:
- 3D-structure
Acetylation
Alternative splicing
Complete proteome
Deafness
Direct protein sequencing
Disease mutation
Fatty acid metabolism
Isomerase
Lipid metabolism
Lyase
NAD
Oxidoreductase
Peroxisome
Phosphoprotein
Polymorphism
Reference proteome