Gene/Proteome Database (LMPD)

LMPD ID
LMP001640
Gene ID
3032
Species
Homo sapiens (Human)
Gene Name
hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit
Gene Symbol
HADHB
Synonyms
ECHB; MTPB; TP-BETA
Chromosome
2
Map Location
2p23
EC Number
2.3.1.16
Summary
This gene encodes the beta subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the beta subunit catalyzing the 3-ketoacyl-CoA thiolase activity. The encoded protein can also bind RNA and decreases the stability of some mRNAs. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. Mutations in this gene result in trifunctional protein deficiency. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2013]
Orthologs
View orthologs and multiple alignments for HADHB

Proteins

trifunctional enzyme subunit beta, mitochondrial isoform 1 precursor
Refseq ID NP_000174
Protein GI 4504327
UniProt ID P55084
mRNA ID NM_000183
Length 474
RefSeq Status REVIEWED
MTILTYPFKNLPTASKWALRFSIRPLSCSSQLRAAPAVQTKTKKTLAKPNIRNVVVVDGVRTPFLLSGTSYKDLMPHDLARAALTGLLHRTSVPKEVVDYIIFGTVIQEVKTSNVAREAALGAGFSDKTPAHTVTMACISANQAMTTGVGLIASGQCDVIVAGGVELMSDVPIRHSRKMRKLMLDLNKAKSMGQRLSLISKFRFNFLAPELPAVSEFSTSETMGHSADRLAAAFAVSRLEQDEYALRSHSLAKKAQDEGLLSDVVPFKVPGKDTVTKDNGIRPSSLEQMAKLKPAFIKPYGTVTAANSSFLTDGASAMLIMAEEKALAMGYKPKAYLRDFMYVSQDPKDQLLLGPTYATPKVLEKAGLTMNDIDAFEFHEAFSGQILANFKAMDSDWFAENYMGRKTKVGLPPLEKFNNWGGSLSLGHPFGATGCRLVMAAANRLRKEGGQYGLVAACAAGGQGHAMIVEAYPK
trifunctional enzyme subunit beta, mitochondrial isoform 2 precursor
Refseq ID NP_001268441
Protein GI 4504327
UniProt ID P55084
mRNA ID NM_001281512
Length 474
RefSeq Status REVIEWED
MTILTYPFKNLPTASKWALRFSIRPLSCSSQLRAAPAVQTKTKKTLAKPNIRNVVVVDGVRTPFLLSGTSYKDLMPHDLARAALTGLLHRTSVPKEVVDYIIFGTVIQEVKTSNVAREAALGAGFSDKTPAHTVTMACISANQAMTTGVGLIASGQCDVIVAGGVELMSDVPIRHSRKMRKLMLDLNKAKSMGQRLSLISKFRFNFLAPELPAVSEFSTSETMGHSADRLAAAFAVSRLEQDEYALRSHSLAKKAQDEGLLSDVVPFKVPGKDTVTKDNGIRPSSLEQMAKLKPAFIKPYGTVTAANSSFLTDGASAMLIMAEEKALAMGYKPKAYLRDFMYVSQDPKDQLLLGPTYATPKVLEKAGLTMNDIDAFEFHEAFSGQILANFKAMDSDWFAENYMGRKTKVGLPPLEKFNNWGGSLSLGHPFGATGCRLVMAAANRLRKEGGQYGLVAACAAGGQGHAMIVEAYPK
trifunctional enzyme subunit beta, mitochondrial isoform 3
Refseq ID NP_001268442
Protein GI 528281413
UniProt ID P55084
mRNA ID NM_001281513
Length 452
RefSeq Status REVIEWED
MTLVSGWLLYGWIIAVQTKTKKTLAKPNIRNVVVVDGVRTPFLLSGTSYKDLMPHDLARAALTGLLHRTSVPKEVVDYIIFGTVIQEVKTSNVAREAALGAGFSDKTPAHTVTMACISANQAMTTGVGLIASGQCDVIVAGGVELMSDVPIRHSRKMRKLMLDLNKAKSMGQRLSLISKFRFNFLAPELPAVSEFSTSETMGHSADRLAAAFAVSRLEQDEYALRSHSLAKKAQDEGLLSDVVPFKVPGKDTVTKDNGIRPSSLEQMAKLKPAFIKPYGTVTAANSSFLTDGASAMLIMAEEKALAMGYKPKAYLRDFMYVSQDPKDQLLLGPTYATPKVLEKAGLTMNDIDAFEFHEAFSGQILANFKAMDSDWFAENYMGRKTKVGLPPLEKFNNWGGSLSLGHPFGATGCRLVMAAANRLRKEGGQYGLVAACAAGGQGHAMIVEAYPK
transit_peptide: 1..33 experiment: experimental evidence, no additional details recorded note: Mitochondrion; propagated from UniProtKB/Swiss-Prot (P55084.3) calculated_mol_wt: 3828 peptide sequence: MTILTYPFKNLPTASKWALRFSIRPLSCSSQLR mat_peptide: 34..474 product: Trifunctional enzyme subunit beta, mitochondrial experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (P55084.3) calculated_mol_wt: 47485 peptide sequence: AAPAVQTKTKKTLAKPNIRNVVVVDGVRTPFLLSGTSYKDLMPHDLARAALTGLLHRTSVPKEVVDYIIFGTVIQEVKTSNVAREAALGAGFSDKTPAHTVTMACISANQAMTTGVGLIASGQCDVIVAGGVELMSDVPIRHSRKMRKLMLDLNKAKSMGQRLSLISKFRFNFLAPELPAVSEFSTSETMGHSADRLAAAFAVSRLEQDEYALRSHSLAKKAQDEGLLSDVVPFKVPGKDTVTKDNGIRPSSLEQMAKLKPAFIKPYGTVTAANSSFLTDGASAMLIMAEEKALAMGYKPKAYLRDFMYVSQDPKDQLLLGPTYATPKVLEKAGLTMNDIDAFEFHEAFSGQILANFKAMDSDWFAENYMGRKTKVGLPPLEKFNNWGGSLSLGHPFGATGCRLVMAAANRLRKEGGQYGLVAACAAGGQGHAMIVEAYPK transit_peptide: 1..33 calculated_mol_wt: 3828 peptide sequence: MTILTYPFKNLPTASKWALRFSIRPLSCSSQLR mat_peptide: 34..459 product: trifunctional enzyme subunit beta, mitochondrial isoform 2 calculated_mol_wt: 45788 peptide sequence: AAPAVQTKTKKTLAKPNIRNVVVVDGVRTPFLLSGTSGLLHRTSVPKEVVDYIIFGTVIQEVKTSNVAREAALGAGFSDKTPAHTVTMACISANQAMTTGVGLIASGQCDVIVAGGVELMSDVPIRHSRKMRKLMLDLNKAKSMGQRLSLISKFRFNFLAPELPAVSEFSTSETMGHSADRLAAAFAVSRLEQDEYALRSHSLAKKAQDEGLLSDVVPFKVPGKDTVTKDNGIRPSSLEQMAKLKPAFIKPYGTVTAANSSFLTDGASAMLIMAEEKALAMGYKPKAYLRDFMYVSQDPKDQLLLGPTYATPKVLEKAGLTMNDIDAFEFHEAFSGQILANFKAMDSDWFAENYMGRKTKVGLPPLEKFNNWGGSLSLGHPFGATGCRLVMAAANRLRKEGGQYGLVAACAAGGQGHAMIVEAYPK

Gene Information

Entrez Gene ID
3032
Gene Name
hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit
Gene Symbol
HADHB
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005783 IDA:UniProtKB C endoplasmic reticulum
GO:0070062 IDA:UniProt C extracellular vesicular exosome
GO:0005740 TAS:ProtInc C mitochondrial envelope
GO:0016507 IEA:Ensembl C mitochondrial fatty acid beta-oxidation multienzyme complex
GO:0005743 IDA:UniProtKB C mitochondrial inner membrane
GO:0042645 IDA:BHF-UCL C mitochondrial nucleoid
GO:0005741 IDA:UniProtKB C mitochondrial outer membrane
GO:0005739 NAS:UniProtKB C mitochondrion
GO:0003857 TAS:ProtInc F 3-hydroxyacyl-CoA dehydrogenase activity
GO:0003988 TAS:ProtInc F acetyl-CoA C-acyltransferase activity
GO:0004300 TAS:ProtInc F enoyl-CoA hydratase activity
GO:0000062 IEA:Ensembl F fatty-acyl-CoA binding
GO:0016509 IEA:Ensembl F long-chain-3-hydroxyacyl-CoA dehydrogenase activity
GO:0016508 IEA:Ensembl F long-chain-enoyl-CoA hydratase activity
GO:0051287 IEA:Ensembl F NAD binding
GO:0044822 IDA:UniProtKB F poly(A) RNA binding
GO:0035965 TAS:Reactome P cardiolipin acyl-chain remodeling
GO:0044255 TAS:Reactome P cellular lipid metabolic process
GO:0006635 TAS:Reactome P fatty acid beta-oxidation
GO:0046474 TAS:Reactome P glycerophospholipid biosynthetic process
GO:0006644 TAS:Reactome P phospholipid metabolic process
GO:0044281 TAS:Reactome P small molecule metabolic process

BIOCYC Pathway Links

BIOCYC Pathway ID Description
PWY66-367 ketogenesis
PWY66-368 ketolysis

REACTOME Pathway Links

REACTOME Pathway ID Description
REACT_121006 Acyl chain remodeling of CL

Domain Information

InterPro Annotations

Accession Description
IPR002155 Thiolase
IPR020610 Thiolase, active site
IPR020615 Thiolase, acyl-enzyme intermediate active site
IPR020613 Thiolase, conserved site
IPR020617 Thiolase, C-terminal
IPR016039 Thiolase-like
IPR016038 Thiolase-like, subgroup
IPR020616 Thiolase, N-terminal

UniProt Annotations

Entry Information

Gene Name
hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit
Protein Entry
ECHB_HUMAN
UniProt ID
P55084
Species
Human

Comments

Comment Type Description
Alternative Products Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=P55084-1; Sequence=Displayed; Name=2; IsoId=P55084-2; Sequence=VSP_054426; Note=No experimental confirmation available.;
Catalytic Activity Acyl-CoA + acetyl-CoA = CoA + 3-oxoacyl-CoA.
Disease Trifunctional protein deficiency (TFP deficiency) [MIM
Interaction Q9H0R8:GABARAPL1; NbExp=4; IntAct=EBI-356635, EBI-746969;
Pathway Lipid metabolism; fatty acid beta-oxidation.
Sequence Caution Sequence=BAA22061.1; Type=Erroneous gene model prediction; Evidence= ;
Similarity Belongs to the thiolase family.
Subcellular Location Mitochondrion . Mitochondrion inner membrane . Mitochondrion outer membrane . Endoplasmic reticulum .
Subunit Octamer of 4 alpha (HADHA) and 4 beta (HADHB) subunits. Interacts with RSAD2/viperin. {ECO

Identical and Related Proteins

Unique RefSeq proteins for LMP001640 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
4504327 RefSeq NP_000174 474 trifunctional enzyme subunit beta, mitochondrial isoform 1 precursor
4504327 RefSeq NP_001268441 474 trifunctional enzyme subunit beta, mitochondrial isoform 2 precursor
528281413 RefSeq NP_001268442 452 trifunctional enzyme subunit beta, mitochondrial isoform 3

Identical Sequences to LMP001640 proteins

Reference Database Accession Length Protein Name
GI:4504327 DBBJ BAG37063.1 474 unnamed protein product [Homo sapiens]
GI:4504327 DBBJ BAG37063.1 474 unnamed protein product [Homo sapiens]
GI:528281413 DBBJ BAG65272.1 452 unnamed protein product [Homo sapiens]
GI:4504327 GenBank ACM80401.1 474 Sequence 5899 from patent US 6812339
GI:4504327 GenBank ACM80401.1 474 Sequence 5899 from patent US 6812339
GI:4504327 GenBank ADF24712.1 474 Sequence 2613 from patent US 7691569
GI:4504327 GenBank ADF24712.1 474 Sequence 2613 from patent US 7691569
GI:4504327 GenBank AED17495.1 474 Sequence 2613 from patent US 7879556
GI:4504327 GenBank AED17495.1 474 Sequence 2613 from patent US 7879556
GI:4504327 GenBank AED47822.1 474 Sequence 2613 from patent US 7892745
GI:4504327 GenBank AED47822.1 474 Sequence 2613 from patent US 7892745
GI:4504327 SwissProt P55084.3 474 RecName: Full=Trifunctional enzyme subunit beta, mitochondrial; AltName: Full=TP-beta; Includes: RecName: Full=3-ketoacyl-CoA thiolase; AltName: Full=Acetyl-CoA acyltransferase; AltName: Full=Beta-ketothiolase; Flags: Precursor [Homo sapiens]
GI:4504327 SwissProt P55084.3 474 RecName: Full=Trifunctional enzyme subunit beta, mitochondrial; AltName: Full=TP-beta; Includes: RecName: Full=3-ketoacyl-CoA thiolase; AltName: Full=Acetyl-CoA acyltransferase; AltName: Full=Beta-ketothiolase; Flags: Precursor [Homo sapiens]

Related Sequences to LMP001640 proteins

Reference Database Accession Length Protein Name
GI:4504327 GenBank AAH17564.1 475 Hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit [Homo sapiens]
GI:4504327 GenBank AAH17564.1 475 Hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit [Homo sapiens]
GI:4504327 GenBank AAH30824.1 475 Hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit [Homo sapiens]
GI:4504327 GenBank AAH30824.1 475 Hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit [Homo sapiens]
GI:4504327 GenBank AAH66963.1 475 Hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit [Homo sapiens]
GI:4504327 GenBank AAH66963.1 475 Hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit [Homo sapiens]
GI:528281413 GenBank ACM80401.1 474 Sequence 5899 from patent US 6812339
GI:4504327 GenBank ADA19618.1 475 Sequence 1442 from patent US 7608413
GI:4504327 GenBank ADA19618.1 475 Sequence 1442 from patent US 7608413
GI:4504327 GenBank AIC58566.1 475 HADHB, partial [synthetic construct]
GI:4504327 GenBank AIC58566.1 475 HADHB, partial [synthetic construct]
GI:528281413 RefSeq NP_000174.1 474 trifunctional enzyme subunit beta, mitochondrial isoform 1 precursor [Homo sapiens]
GI:528281413 RefSeq XP_003270654.1 452 PREDICTED: trifunctional enzyme subunit beta, mitochondrial isoform 2 [Nomascus leucogenys]
GI:528281413 RefSeq XP_004029005.1 452 PREDICTED: trifunctional enzyme subunit beta, mitochondrial isoform 2 [Gorilla gorilla gorilla]
GI:528281413 RefSeq XP_005576412.1 459 PREDICTED: trifunctional enzyme subunit beta, mitochondrial isoform X5 [Macaca fascicularis]
GI:4504327 RefSeq XP_009440359.1 475 PREDICTED: trifunctional enzyme subunit beta, mitochondrial isoform X1 [Pan troglodytes]
GI:4504327 RefSeq XP_009440359.1 475 PREDICTED: trifunctional enzyme subunit beta, mitochondrial isoform X1 [Pan troglodytes]
GI:528281413 SwissProt P55084.3 474 RecName: Full=Trifunctional enzyme subunit beta, mitochondrial; AltName: Full=TP-beta; Includes: RecName: Full=3-ketoacyl-CoA thiolase; AltName: Full=Acetyl-CoA acyltransferase; AltName: Full=Beta-ketothiolase; Flags: Precursor [Homo sapiens]