Entity Details

Primary name DHB4_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionP51659
EntryNameDHB4_HUMAN
FullNamePeroxisomal multifunctional enzyme type 2
TaxID9606
Evidenceevidence at protein level
Length736
SequenceStatuscomplete
DateCreated1996-10-01
DateModified2021-06-02

Ontological Relatives

GenesHSD17B4

GO terms

Show/Hide Table
GOName
GO:0000038 very long-chain fatty acid metabolic process
GO:0001649 osteoblast differentiation
GO:0003857 3-hydroxyacyl-CoA dehydrogenase activity
GO:0004300 enoyl-CoA hydratase activity
GO:0005777 peroxisome
GO:0005778 peroxisomal membrane
GO:0005782 peroxisomal matrix
GO:0005829 cytosol
GO:0006635 fatty acid beta-oxidation
GO:0006699 bile acid biosynthetic process
GO:0008104 protein localization
GO:0008209 androgen metabolic process
GO:0008210 estrogen metabolic process
GO:0016020 membrane
GO:0016508 long-chain-enoyl-CoA hydratase activity
GO:0016853 isomerase activity
GO:0033540 fatty acid beta-oxidation using acyl-CoA oxidase
GO:0033989 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA hydratase activity
GO:0036109 alpha-linolenic acid metabolic process
GO:0036111 very long-chain fatty-acyl-CoA metabolic process
GO:0036112 medium-chain fatty-acyl-CoA metabolic process
GO:0042803 protein homodimerization activity
GO:0044594 17-beta-hydroxysteroid dehydrogenase (NAD+) activity
GO:0060009 Sertoli cell development

Subcellular Location

Show/Hide Table
Subcellular Location
Peroxisome

Domains

Show/Hide Table
DomainNameCategoryType
IPR002347 Short-chain dehydrogenase/reductase SDRFamilyFamily
IPR002539 MaoC-like dehydratase domainDomainDomain
IPR003033 SCP2 sterol-binding domainDomainDomain
IPR020904 Short-chain dehydrogenase/reductase, conserved siteSiteConserved site
IPR029069 HotDog domain superfamilyFamilyHomologous superfamily
IPR036291 NAD(P)-binding domain superfamilyFamilyHomologous superfamily
IPR036527 SCP2 sterol-binding domain superfamilyFamilyHomologous superfamily

Diseases

Show/Hide Table
Disease IDSourceNameDescription
233400 OMIMPerrault syndrome 1 (PRLTS1)A sex-influenced disorder characterized by sensorineural deafness in both males and females and ovarian dysgenesis in females. Some patients also have neurologic manifestations, including mild mental retardation and cerebellar and peripheral nervous system involvement. The disease is caused by variants affecting the gene represented in this entry.
261515 OMIMD-bifunctional protein deficiency (DBPD)Disorder of peroxisomal fatty acid beta-oxidation. The disease is caused by variants affecting the gene represented in this entry.

Drugs

Show/Hide Table
DrugNameSourceType
DB00157 NADHDrugbanksmall molecule
DB03192 (R)-3-hydroxydecanoyl-CoADrugbanksmall molecule