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
REACTOME Pathway Links
REACTOME Pathway ID | Description |
---|---|
REACT_121147 | alpha-linolenic acid (ALA) metabolism |
Domain Information
InterPro Annotations
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] |