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Protein

Peroxisomal bifunctional enzyme

Gene

Ehhadh

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Catalytic activityi

(3S)-3-hydroxyacyl-CoA = trans-2(or 3)-enoyl-CoA + H2O.
A (3Z)-alk-3-enoyl-CoA = a (2E)-alk-2-enoyl-CoA.
A (3E)-alk-3-enoyl-CoA = a (2E)-alk-2-enoyl-CoA.
(S)-3-hydroxyacyl-CoA + NAD+ = 3-oxoacyl-CoA + NADH.

Enzyme regulationi

Enzyme activity enhanced by acetylation.By similarity

Pathwayi: fatty acid beta-oxidation

This protein is involved in the pathway fatty acid beta-oxidation, which is part of Lipid metabolism.
View all proteins of this organism that are known to be involved in the pathway fatty acid beta-oxidation and in Lipid metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei99Substrate; via amide nitrogenBy similarity1
Sitei102Important for catalytic activityBy similarity1
Sitei122Important for catalytic activityBy similarity1

GO - Molecular functioni

GO - Biological processi

  • acyl-CoA metabolic process Source: MGI
  • fatty acid beta-oxidation Source: MGI
  • internal protein amino acid acetylation Source: UniProtKB

Keywordsi

Molecular functionIsomerase, Lyase, Multifunctional enzyme, Oxidoreductase
Biological processFatty acid metabolism, Lipid metabolism
LigandNAD

Enzyme and pathway databases

ReactomeiR-MMU-390247 Beta-oxidation of very long chain fatty acids
UniPathwayiUPA00659

Chemistry databases

SwissLipidsiSLP:000000402
SLP:000000491

Names & Taxonomyi

Protein namesi
Recommended name:
Peroxisomal bifunctional enzyme
Short name:
PBE
Short name:
PBFE
Including the following 2 domains:
Enoyl-CoA hydratase/3,2-trans-enoyl-CoA isomerase (EC:4.2.1.17, EC:5.3.3.8)
3-hydroxyacyl-CoA dehydrogenase (EC:1.1.1.35)
Gene namesi
Name:Ehhadh
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 16

Organism-specific databases

MGIiMGI:1277964 Ehhadh

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Peroxisome

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00001092481 – 718Peroxisomal bifunctional enzymeAdd BLAST718

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei38N6-succinyllysineCombined sources1
Modified residuei163N6-acetyllysine; alternateBy similarity1
Modified residuei163N6-succinyllysine; alternateCombined sources1
Modified residuei172N6-acetyllysine; alternateCombined sources1
Modified residuei172N6-succinyllysine; alternateCombined sources1
Modified residuei181N6-succinyllysineCombined sources1
Modified residuei189N6-acetyllysine; alternateCombined sources1
Modified residuei189N6-succinyllysine; alternateCombined sources1
Modified residuei217N6-acetyllysine; alternateCombined sources1
Modified residuei217N6-succinyllysine; alternateCombined sources1
Modified residuei240N6-succinyllysineCombined sources1
Modified residuei248N6-acetyllysineCombined sources1
Modified residuei252N6-succinyllysineCombined sources1
Modified residuei274N6-acetyllysine; alternateCombined sources1
Modified residuei274N6-succinyllysine; alternateCombined sources1
Modified residuei278N6-succinyllysineCombined sources1
Modified residuei288N6-succinyllysineCombined sources1
Modified residuei329N6-succinyllysineCombined sources1
Modified residuei344N6-acetyllysineCombined sources1
Modified residuei348N6-acetyllysineCombined sources1
Modified residuei355N6-acetyllysineCombined sources1
Modified residuei459N6-acetyllysineCombined sources1
Modified residuei527N6-succinyllysineCombined sources1
Modified residuei543PhosphothreonineCombined sources1
Modified residuei572N6-succinyllysineCombined sources1
Modified residuei579N6-acetyllysine; alternateCombined sources1
Modified residuei579N6-succinyllysine; alternateCombined sources1
Modified residuei586N6-acetyllysine; alternateCombined sources1
Modified residuei586N6-succinyllysine; alternateCombined sources1
Modified residuei705N6-acetyllysine; alternateCombined sources1
Modified residuei705N6-succinyllysine; alternateCombined sources1
Modified residuei717N6-succinyllysineCombined sources1

Post-translational modificationi

Acetylated, leading to enhanced enzyme activity. Acetylation is enhanced by up to 80% after treatment either with trichostin A (TCA) or with nicotinamide (NAM) with highest increase on Lys-344. Acetylation and enzyme activity increased by about 1.5% on addition of fatty acids (By similarity).By similarity

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiQ9DBM2
MaxQBiQ9DBM2
PaxDbiQ9DBM2
PeptideAtlasiQ9DBM2
PRIDEiQ9DBM2

PTM databases

iPTMnetiQ9DBM2
PhosphoSitePlusiQ9DBM2
SwissPalmiQ9DBM2

Expressioni

Gene expression databases

BgeeiENSMUSG00000022853
GenevisibleiQ9DBM2 MM

Interactioni

Subunit structurei

Monomer.By similarity

GO - Molecular functioni

Protein-protein interaction databases

IntActiQ9DBM2, 5 interactors
MINTiQ9DBM2
STRINGi10090.ENSMUSP00000023559

Structurei

3D structure databases

ProteinModelPortaliQ9DBM2
SMRiQ9DBM2
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni1 – 280Enoyl-CoA hydratase / isomeraseAdd BLAST280
Regioni281 – 5673-hydroxyacyl-CoA dehydrogenaseAdd BLAST287

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi716 – 718Microbody targeting signalBy similarity3

Sequence similaritiesi

In the N-terminal section; belongs to the enoyl-CoA hydratase/isomerase family.Curated
In the C-terminal section; belongs to the 3-hydroxyacyl-CoA dehydrogenase family.Curated

Phylogenomic databases

eggNOGiKOG1683 Eukaryota
COG1250 LUCA
GeneTreeiENSGT00880000137923
HOGENOMiHOG000261347
HOVERGENiHBG104990
InParanoidiQ9DBM2
KOiK07514
OMAiIDLCMIL
OrthoDBiEOG091G082G
TreeFamiTF316708

Family and domain databases

InterProiView protein in InterPro
IPR006180 3-OHacyl-CoA_DH_CS
IPR006176 3-OHacyl-CoA_DH_NAD-bd
IPR006108 3HC_DH_C
IPR008927 6-PGluconate_DH-like_C_sf
IPR029045 ClpP/crotonase-like_dom_sf
IPR018376 Enoyl-CoA_hyd/isom_CS
IPR001753 Enoyl-CoA_hydra/iso
IPR036291 NAD(P)-bd_dom_sf
PfamiView protein in Pfam
PF00725 3HCDH, 2 hits
PF02737 3HCDH_N, 1 hit
PF00378 ECH_1, 1 hit
SUPFAMiSSF48179 SSF48179, 2 hits
SSF51735 SSF51735, 1 hit
SSF52096 SSF52096, 1 hit
PROSITEiView protein in PROSITE
PS00067 3HCDH, 1 hit
PS00166 ENOYL_COA_HYDRATASE, 1 hit

Sequencei

Sequence statusi: Complete.

Q9DBM2-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAEYLRLPHS LAMIRLCNPP VNAISPTVIT EVRNGLQKAS LDHTVRAIVI
60 70 80 90 100
CGANDNFCAG ADIHGFKSPT GLTLGSLVDE IQRYQKPVVA AIQGVALGGG
110 120 130 140 150
LELALGCHYR IANAKARVGF PEVMLGILPG ARGTQLLPRV VGVPVALDLI
160 170 180 190 200
TSGRHISTDE ALKLGILDVV VKSDPVEEAI KFAQTVIGKP IEPRRILNKP
210 220 230 240 250
VPSLPNMDSV FAEAIAKVRK QYPGRLAPET CVRSVQASVK HPYEVAIKEE
260 270 280 290 300
AKLFMYLRGS GQARALQYAF FAEKSANKWS TPSGASWKTA SAQPVSSVGV
310 320 330 340 350
LGLGTMGRGI AISFARVGIP VVAVESDPKQ LDTAKKIITS TLEKEASKSG
360 370 380 390 400
QASAKPNLRF SSSTKELSSV DLVIEAVFED MNLKKKVFAE LSALCKPGAF
410 420 430 440 450
LCTNTSALDV DDIASSTDRP QLVIGTHFFS PAHIMRLLEV IPSRYSSPTT
460 470 480 490 500
IATVMSLSKR IGKIGVVVGN CYGFVGNRML APYYNQGYFL IEEGSKPEDV
510 520 530 540 550
DGVLEEFGFR MGPFRVSDLA GLDVGWKVRK GQGLTGPSLP PGTPTRKRGN
560 570 580 590 600
TRYSPIADML CEAGRFGQKT GKGWYQYDKP LGRIHKPDPW LSEFLSQYRE
610 620 630 640 650
THHIKQRSIS KEEILERCLY SLINEAFRIL EEGMAASPEH IDVIYLHGYG
660 670 680 690 700
WPRHVGGPMY YAASVGLPTV LEKLQKYYRQ NPDIPQLEPS DYLRRLVAQG
710
SPPLKEWQSL AGPHSSKL
Length:718
Mass (Da):78,301
Last modified:July 27, 2011 - v4
Checksum:iA405717EC7A3EBD9
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti499D → G in BAB23628 (PubMed:16141072).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK004867 mRNA Translation: BAB23628.1
BC016899 mRNA Translation: AAH16899.1
CH466521 Genomic DNA Translation: EDK97607.1
CCDSiCCDS28063.1
RefSeqiNP_076226.2, NM_023737.3
UniGeneiMm.28100

Genome annotation databases

EnsembliENSMUST00000023559; ENSMUSP00000023559; ENSMUSG00000022853
GeneIDi74147
KEGGimmu:74147
UCSCiuc007yrm.2 mouse

Similar proteinsi

Entry informationi

Entry nameiECHP_MOUSE
AccessioniPrimary (citable) accession number: Q9DBM2
Secondary accession number(s): Q91W49
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 24, 2001
Last sequence update: July 27, 2011
Last modified: April 25, 2018
This is version 154 of the entry and version 4 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health