Gene/Proteome Database (LMPD)

LMPD ID
LMP000062
Gene ID
Species
Homo sapiens (Human)
Gene Name
hydroxysteroid (17-beta) dehydrogenase 4
Gene Symbol
Synonyms
DBP; MFE-2; MPF-2; PRLTS1; SDR8C1
Chromosome
5
Map Location
5q21
Summary
The protein encoded by this gene is a bifunctional enzyme that is involved in the peroxisomal beta-oxidation pathway for fatty acids. It also acts as a catalyst for the formation of 3-ketoacyl-CoA intermediates from both straight-chain and 2-methyl-branched-chain fatty acids. Defects in this gene that affect the peroxisomal fatty acid beta-oxidation activity are a cause of D-bifunctional protein deficiency (DBPD). An apparent pseudogene of this gene is present on chromosome 8. Multiple alternatively spliced transcript variants encoding distinct isoforms have been found for this gene. [provided by RefSeq, May 2014]
Orthologs

Proteins

peroxisomal multifunctional enzyme type 2 isoform 1
Refseq ID NP_001186220
Protein GI 4504505
UniProt ID P51659
mRNA ID NM_001199291
Length 736
RefSeq Status REVIEWED
MGSPLRFDGRVVLVTGAGAGLGRAYALAFAERGALVVVNDLGGDFKGVGKGSLAADKVVEEIRRRGGKAVANYDSVEEGEKVVKTALDAFGRIDVVVNNAGILRDRSFARISDEDWDIIHRVHLRGSFQVTRAAWEHMKKQKYGRIIMTSSASGIYGNFGQANYSAAKLGLLGLANSLAIEGRKSNIHCNTIAPNAGSRMTQTVMPEDLVEALKPEYVAPLVLWLCHESCEENGGLFEVGAGWIGKLRWERTLGAIVRQKNHPMTPEAVKANWKKICDFENASKPQSIQESTGSIIEVLSKIDSEGGVSANHTSRATSTATSGFAGAIGQKLPPFSYAYTELEAIMYALGVGASIKDPKDLKFIYEGSSDFSCLPTFGVIIGQKSMMGGGLAEIPGLSINFAKVLHGEQYLELYKPLPRAGKLKCEAVVADVLDKGSGVVIIMDVYSYSEKELICHNQFSLFLVGSGGFGGKRTSDKVKVAVAIPNRPPDAVLTDTTSLNQAALYRLSGDWNPLHIDPNFASLAGFDKPILHGLCTFGFSARRVLQQFADNDVSRFKAIKARFAKPVYPGQTLQTEMWKEGNRIHFQTKVQETGDIVISNAYVDLAPTSGTSAKTPSEGGKLQSTFVFEEIGRRLKDIGPEVVKKVNAVFEWHITKGGNIGAKWTIDLKSGSGKVYQGPAKGAADTTIILSDEDFMEVVLGKLDPQKAFFSGRLKARGNIMLSQKLQMILKDYAKL
peroxisomal multifunctional enzyme type 2 isoform 2
Refseq ID NP_000405
Protein GI 4504505
UniProt ID P51659
mRNA ID NM_000414
Length 736
RefSeq Status REVIEWED
MGSPLRFDGRVVLVTGAGAGLGRAYALAFAERGALVVVNDLGGDFKGVGKGSLAADKVVEEIRRRGGKAVANYDSVEEGEKVVKTALDAFGRIDVVVNNAGILRDRSFARISDEDWDIIHRVHLRGSFQVTRAAWEHMKKQKYGRIIMTSSASGIYGNFGQANYSAAKLGLLGLANSLAIEGRKSNIHCNTIAPNAGSRMTQTVMPEDLVEALKPEYVAPLVLWLCHESCEENGGLFEVGAGWIGKLRWERTLGAIVRQKNHPMTPEAVKANWKKICDFENASKPQSIQESTGSIIEVLSKIDSEGGVSANHTSRATSTATSGFAGAIGQKLPPFSYAYTELEAIMYALGVGASIKDPKDLKFIYEGSSDFSCLPTFGVIIGQKSMMGGGLAEIPGLSINFAKVLHGEQYLELYKPLPRAGKLKCEAVVADVLDKGSGVVIIMDVYSYSEKELICHNQFSLFLVGSGGFGGKRTSDKVKVAVAIPNRPPDAVLTDTTSLNQAALYRLSGDWNPLHIDPNFASLAGFDKPILHGLCTFGFSARRVLQQFADNDVSRFKAIKARFAKPVYPGQTLQTEMWKEGNRIHFQTKVQETGDIVISNAYVDLAPTSGTSAKTPSEGGKLQSTFVFEEIGRRLKDIGPEVVKKVNAVFEWHITKGGNIGAKWTIDLKSGSGKVYQGPAKGAADTTIILSDEDFMEVVLGKLDPQKAFFSGRLKARGNIMLSQKLQMILKDYAKL
peroxisomal multifunctional enzyme type 2 isoform 3
Refseq ID NP_001186221
Protein GI 4504505
UniProt ID P51659
mRNA ID NM_001199292
Length 736
RefSeq Status REVIEWED
MGSPLRFDGRVVLVTGAGAGLGRAYALAFAERGALVVVNDLGGDFKGVGKGSLAADKVVEEIRRRGGKAVANYDSVEEGEKVVKTALDAFGRIDVVVNNAGILRDRSFARISDEDWDIIHRVHLRGSFQVTRAAWEHMKKQKYGRIIMTSSASGIYGNFGQANYSAAKLGLLGLANSLAIEGRKSNIHCNTIAPNAGSRMTQTVMPEDLVEALKPEYVAPLVLWLCHESCEENGGLFEVGAGWIGKLRWERTLGAIVRQKNHPMTPEAVKANWKKICDFENASKPQSIQESTGSIIEVLSKIDSEGGVSANHTSRATSTATSGFAGAIGQKLPPFSYAYTELEAIMYALGVGASIKDPKDLKFIYEGSSDFSCLPTFGVIIGQKSMMGGGLAEIPGLSINFAKVLHGEQYLELYKPLPRAGKLKCEAVVADVLDKGSGVVIIMDVYSYSEKELICHNQFSLFLVGSGGFGGKRTSDKVKVAVAIPNRPPDAVLTDTTSLNQAALYRLSGDWNPLHIDPNFASLAGFDKPILHGLCTFGFSARRVLQQFADNDVSRFKAIKARFAKPVYPGQTLQTEMWKEGNRIHFQTKVQETGDIVISNAYVDLAPTSGTSAKTPSEGGKLQSTFVFEEIGRRLKDIGPEVVKKVNAVFEWHITKGGNIGAKWTIDLKSGSGKVYQGPAKGAADTTIILSDEDFMEVVLGKLDPQKAFFSGRLKARGNIMLSQKLQMILKDYAKL
peroxisomal multifunctional enzyme type 2 isoform 4
Refseq ID NP_001278956
Protein GI 4504505
UniProt ID P51659
mRNA ID NM_001292027
Length 736
RefSeq Status REVIEWED
MGSPLRFDGRVVLVTGAGAGLGRAYALAFAERGALVVVNDLGGDFKGVGKGSLAADKVVEEIRRRGGKAVANYDSVEEGEKVVKTALDAFGRIDVVVNNAGILRDRSFARISDEDWDIIHRVHLRGSFQVTRAAWEHMKKQKYGRIIMTSSASGIYGNFGQANYSAAKLGLLGLANSLAIEGRKSNIHCNTIAPNAGSRMTQTVMPEDLVEALKPEYVAPLVLWLCHESCEENGGLFEVGAGWIGKLRWERTLGAIVRQKNHPMTPEAVKANWKKICDFENASKPQSIQESTGSIIEVLSKIDSEGGVSANHTSRATSTATSGFAGAIGQKLPPFSYAYTELEAIMYALGVGASIKDPKDLKFIYEGSSDFSCLPTFGVIIGQKSMMGGGLAEIPGLSINFAKVLHGEQYLELYKPLPRAGKLKCEAVVADVLDKGSGVVIIMDVYSYSEKELICHNQFSLFLVGSGGFGGKRTSDKVKVAVAIPNRPPDAVLTDTTSLNQAALYRLSGDWNPLHIDPNFASLAGFDKPILHGLCTFGFSARRVLQQFADNDVSRFKAIKARFAKPVYPGQTLQTEMWKEGNRIHFQTKVQETGDIVISNAYVDLAPTSGTSAKTPSEGGKLQSTFVFEEIGRRLKDIGPEVVKKVNAVFEWHITKGGNIGAKWTIDLKSGSGKVYQGPAKGAADTTIILSDEDFMEVVLGKLDPQKAFFSGRLKARGNIMLSQKLQMILKDYAKL
peroxisomal multifunctional enzyme type 2 isoform 5
Refseq ID NP_001278957
Protein GI 635372938
UniProt ID P51659
mRNA ID NM_001292028
Length 596
RefSeq Status REVIEWED
MKKQKIIMTSSASGIYGNFGQANYSAAKLGLLGLANSLAIEGRKSNIHCNTIAPNAGSRMTQTVMPEDLVEALKPEYVAPLVLWLCHESCEENGGLFEVGAGWIGKLRWERTLGAIVRQKNHPMTPEAVKANWKKICDFENASKPQSIQESTGSIIEVLSKIDSEGGVSANHTSRATSTATSGFAGAIGQKLPPFSYAYTELEAIMYALGVGASIKDPKDLKFIYEGSSDFSCLPTFGVIIGQKSMMGGGLAEIPGLSINFAKVLHGEQYLELYKPLPRAGKLKCEAVVADVLDKGSGVVIIMDVYSYSEKELICHNQFSLFLVGSGGFGGKRTSDKVKVAVAIPNRPPDAVLTDTTSLNQAALYRLSGDWNPLHIDPNFASLAGFDKPILHGLCTFGFSARRVLQQFADNDVSRFKAIKARFAKPVYPGQTLQTEMWKEGNRIHFQTKVQETGDIVISNAYVDLAPTSGTSAKTPSEGGKLQSTFVFEEIGRRLKDIGPEVVKKVNAVFEWHITKGGNIGAKWTIDLKSGSGKVYQGPAKGAADTTIILSDEDFMEVVLGKLDPQKAFFSGRLKARGNIMLSQKLQMILKDYAKL
mat_peptide: 1..311 product: (3R)-hydroxyacyl-CoA dehydrogenase experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (P51659.3) calculated_mol_wt: 33582 peptide sequence: MGSPLRFDGRVVLVTGAGAGLGRAYALAFAERGALVVVNDLGGDFKGVGKGSLAADKVVEEIRRRGGKAVANYDSVEEGEKVVKTALDAFGRIDVVVNNAGILRDRSFARISDEDWDIIHRVHLRGSFQVTRAAWEHMKKQKYGRIIMTSSASGIYGNFGQANYSAAKLGLLGLANSLAIEGRKSNIHCNTIAPNAGSRMTQTVMPEDLVEALKPEYVAPLVLWLCHESCEENGGLFEVGAGWIGKLRWERTLGAIVRQKNHPMTPEAVKANWKKICDFENASKPQSIQESTGSIIEVLSKIDSEGGVSAN mat_peptide: 312..736 product: Enoyl-CoA hydratase 2 experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (P51659.3) calculated_mol_wt: 46122 peptide sequence: HTSRATSTATSGFAGAIGQKLPPFSYAYTELEAIMYALGVGASIKDPKDLKFIYEGSSDFSCLPTFGVIIGQKSMMGGGLAEIPGLSINFAKVLHGEQYLELYKPLPRAGKLKCEAVVADVLDKGSGVVIIMDVYSYSEKELICHNQFSLFLVGSGGFGGKRTSDKVKVAVAIPNRPPDAVLTDTTSLNQAALYRLSGDWNPLHIDPNFASLAGFDKPILHGLCTFGFSARRVLQQFADNDVSRFKAIKARFAKPVYPGQTLQTEMWKEGNRIHFQTKVQETGDIVISNAYVDLAPTSGTSAKTPSEGGKLQSTFVFEEIGRRLKDIGPEVVKKVNAVFEWHITKGGNIGAKWTIDLKSGSGKVYQGPAKGAADTTIILSDEDFMEVVLGKLDPQKAFFSGRLKARGNIMLSQKLQMILKDYAKL

Gene Information

Entrez Gene ID
Gene Name
hydroxysteroid (17-beta) dehydrogenase 4
Gene Symbol
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0016491 IEA:InterPro F oxidoreductase activity

KEGG Pathway Links

KEGG Pathway ID Description
hsa04146 Peroxisome
hsa00120 Primary bile acid biosynthesis

REACTOME Pathway Links

REACTOME Pathway ID Description
REACT_121147 alpha-linolenic acid (ALA) metabolism

Domain Information

InterPro Annotations

Accession Description
IPR002347 Glucose/ribitol dehydrogenase
IPR029069 HotDog domain
IPR002539 MaoC-like domain
IPR016040 NAD(P)-binding domain
IPR003033 SCP2 sterol-binding domain
IPR002198 Short-chain dehydrogenase/reductase SDR
IPR020904 Short-chain dehydrogenase/reductase, conserved site

UniProt Annotations

Entry Information

Gene Name
hydroxysteroid (17-beta) dehydrogenase 4
Protein Entry
B2R659_HUMAN
UniProt ID
Species
Human

Identical and Related Proteins

Unique RefSeq proteins for LMP000062 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
4504505 RefSeq NP_001186220 736 peroxisomal multifunctional enzyme type 2 isoform 1
4504505 RefSeq NP_000405 736 peroxisomal multifunctional enzyme type 2 isoform 2
4504505 RefSeq NP_001186221 736 peroxisomal multifunctional enzyme type 2 isoform 3
4504505 RefSeq NP_001278956 736 peroxisomal multifunctional enzyme type 2 isoform 4
635372938 RefSeq NP_001278957 596 peroxisomal multifunctional enzyme type 2 isoform 5

Identical Sequences to LMP000062 proteins

Reference Database Accession Length Protein Name
GI:635372938 GenBank EAW48910.1 596 hydroxysteroid (17-beta) dehydrogenase 4, isoform CRA_a [Homo sapiens]
GI:4504505 GenBank ABM82792.1 736 hydroxysteroid (17-beta) dehydrogenase 4 [synthetic construct]
GI:4504505 GenBank ABM82792.1 736 hydroxysteroid (17-beta) dehydrogenase 4 [synthetic construct]
GI:4504505 GenBank ABM82792.1 736 hydroxysteroid (17-beta) dehydrogenase 4 [synthetic construct]
GI:4504505 GenBank ABM82792.1 736 hydroxysteroid (17-beta) dehydrogenase 4 [synthetic construct]
GI:4504505 GenBank ABM85981.1 736 hydroxysteroid (17-beta) dehydrogenase 4, partial [synthetic construct]
GI:4504505 GenBank ABM85981.1 736 hydroxysteroid (17-beta) dehydrogenase 4, partial [synthetic construct]
GI:4504505 GenBank ABM85981.1 736 hydroxysteroid (17-beta) dehydrogenase 4, partial [synthetic construct]
GI:4504505 GenBank ABM85981.1 736 hydroxysteroid (17-beta) dehydrogenase 4, partial [synthetic construct]
GI:635372938 GenBank ADT50450.1 596 Sequence 2467 from patent US 7842467
GI:4504505 GenBank ADT50451.1 736 Sequence 2468 from patent US 7842467
GI:4504505 GenBank ADT50451.1 736 Sequence 2468 from patent US 7842467
GI:4504505 GenBank ADT50451.1 736 Sequence 2468 from patent US 7842467
GI:4504505 GenBank ADT50451.1 736 Sequence 2468 from patent US 7842467
GI:4504505 GenBank ADT50452.1 736 Sequence 2469 from patent US 7842467
GI:4504505 GenBank ADT50452.1 736 Sequence 2469 from patent US 7842467
GI:4504505 GenBank ADT50452.1 736 Sequence 2469 from patent US 7842467
GI:4504505 GenBank ADT50452.1 736 Sequence 2469 from patent US 7842467
GI:4504505 GenBank AHD74596.1 736 Sequence 14613 from patent US 8586006
GI:4504505 GenBank AHD74596.1 736 Sequence 14613 from patent US 8586006
GI:4504505 GenBank AHD74596.1 736 Sequence 14613 from patent US 8586006
GI:4504505 GenBank AHD74596.1 736 Sequence 14613 from patent US 8586006
GI:4504505 GenBank AIC48986.1 736 HSD17B4, partial [synthetic construct]
GI:4504505 GenBank AIC48986.1 736 HSD17B4, partial [synthetic construct]
GI:4504505 GenBank AIC48986.1 736 HSD17B4, partial [synthetic construct]
GI:4504505 GenBank AIC48986.1 736 HSD17B4, partial [synthetic construct]

Related Sequences to LMP000062 proteins

Reference Database Accession Length Protein Name
GI:4504505 DBBJ BAG35356.1 736 unnamed protein product [Homo sapiens]
GI:4504505 DBBJ BAG35356.1 736 unnamed protein product [Homo sapiens]
GI:4504505 DBBJ BAG35356.1 736 unnamed protein product [Homo sapiens]
GI:4504505 DBBJ BAG35356.1 736 unnamed protein product [Homo sapiens]
GI:635372938 DBBJ BAG52804.1 596 unnamed protein product [Homo sapiens]
GI:4504505 EMBL CAC07797.1 735 unnamed protein product [unidentified]
GI:4504505 EMBL CAC07797.1 735 unnamed protein product [unidentified]
GI:4504505 EMBL CAC07797.1 735 unnamed protein product [unidentified]
GI:4504505 EMBL CAC07797.1 735 unnamed protein product [unidentified]
GI:635372938 GenBank AAD08652.1 736 17-beta-hydroxysteroid dehydrogenase IV [Homo sapiens]
GI:635372938 GenBank ABM82792.1 736 hydroxysteroid (17-beta) dehydrogenase 4 [synthetic construct]
GI:635372938 GenBank ABM85981.1 736 hydroxysteroid (17-beta) dehydrogenase 4, partial [synthetic construct]
GI:4504505 GenBank JAA09354.1 736 hydroxysteroid (17-beta) dehydrogenase 4 [Pan troglodytes]
GI:4504505 GenBank JAA09354.1 736 hydroxysteroid (17-beta) dehydrogenase 4 [Pan troglodytes]
GI:4504505 GenBank JAA09354.1 736 hydroxysteroid (17-beta) dehydrogenase 4 [Pan troglodytes]
GI:4504505 GenBank JAA09354.1 736 hydroxysteroid (17-beta) dehydrogenase 4 [Pan troglodytes]
GI:4504505 GenBank JAA23146.1 736 hydroxysteroid (17-beta) dehydrogenase 4 [Pan troglodytes]
GI:4504505 GenBank JAA23146.1 736 hydroxysteroid (17-beta) dehydrogenase 4 [Pan troglodytes]
GI:4504505 GenBank JAA23146.1 736 hydroxysteroid (17-beta) dehydrogenase 4 [Pan troglodytes]
GI:4504505 GenBank JAA23146.1 736 hydroxysteroid (17-beta) dehydrogenase 4 [Pan troglodytes]
GI:635372938 GenBank AHD74596.1 736 Sequence 14613 from patent US 8586006
GI:635372938 RefSeq NP_000405.1 736 peroxisomal multifunctional enzyme type 2 isoform 2 [Homo sapiens]
GI:4504505 RefSeq XP_003310890.1 736 PREDICTED: peroxisomal multifunctional enzyme type 2 isoform X1 [Pan troglodytes]
GI:4504505 RefSeq XP_003310890.1 736 PREDICTED: peroxisomal multifunctional enzyme type 2 isoform X1 [Pan troglodytes]
GI:4504505 RefSeq XP_003310890.1 736 PREDICTED: peroxisomal multifunctional enzyme type 2 isoform X1 [Pan troglodytes]
GI:4504505 RefSeq XP_003310890.1 736 PREDICTED: peroxisomal multifunctional enzyme type 2 isoform X1 [Pan troglodytes]
GI:4504505 RefSeq XP_003826755.1 736 PREDICTED: peroxisomal multifunctional enzyme type 2 isoform X2 [Pan paniscus]
GI:4504505 RefSeq XP_003826755.1 736 PREDICTED: peroxisomal multifunctional enzyme type 2 isoform X2 [Pan paniscus]
GI:4504505 RefSeq XP_003826755.1 736 PREDICTED: peroxisomal multifunctional enzyme type 2 isoform X2 [Pan paniscus]
GI:4504505 RefSeq XP_003826755.1 736 PREDICTED: peroxisomal multifunctional enzyme type 2 isoform X2 [Pan paniscus]