Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Delta(3,5)-Delta(2,4)-dienoyl-CoA isomerase, mitochondrial

Gene

ECH1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Isomerization of 3-trans,5-cis-dienoyl-CoA to 2-trans,4-trans-dienoyl-CoA.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 sitei174Substrate; via amide nitrogenBy similarity1
Sitei197Important for catalytic activityBy similarity1
Sitei205Important for catalytic activityBy similarity1

GO - Molecular functioni

  • isomerase activity Source: UniProtKB-KW
  • receptor binding Source: UniProtKB

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Isomerase

Keywords - Biological processi

Fatty acid metabolism, Lipid metabolism

Enzyme and pathway databases

BioCyciZFISH:ENSG00000104823-MONOMER.
UniPathwayiUPA00659.

Names & Taxonomyi

Protein namesi
Recommended name:
Delta(3,5)-Delta(2,4)-dienoyl-CoA isomerase, mitochondrial (EC:5.3.3.-)
Gene namesi
Name:ECH1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 19

Organism-specific databases

HGNCiHGNC:3149. ECH1.

Subcellular locationi

GO - Cellular componenti

  • extracellular exosome Source: UniProtKB
  • membrane Source: UniProtKB
  • mitochondrion Source: GO_Central
  • peroxisome Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion, Peroxisome

Pathology & Biotechi

Organism-specific databases

OpenTargetsiENSG00000104823.
ENSG00000282853.
PharmGKBiPA27596.

Polymorphism and mutation databases

BioMutaiECH1.
DMDMi82654933.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 33MitochondrionSequence analysisAdd BLAST33
ChainiPRO_000000741734 – 328Delta(3,5)-Delta(2,4)-dienoyl-CoA isomerase, mitochondrialAdd BLAST295

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei231N6-succinyllysineBy similarity1
Modified residuei268PhosphoserineCombined sources1
Modified residuei327N6-acetyllysineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiQ13011.
MaxQBiQ13011.
PaxDbiQ13011.
PeptideAtlasiQ13011.
PRIDEiQ13011.
TopDownProteomicsiQ13011.

2D gel databases

REPRODUCTION-2DPAGEIPI00011416.
UCD-2DPAGEQ13011.

PTM databases

iPTMnetiQ13011.
PhosphoSitePlusiQ13011.
SwissPalmiQ13011.

Expressioni

Gene expression databases

BgeeiENSG00000104823.
CleanExiHS_ECH1.
ExpressionAtlasiQ13011. baseline and differential.
GenevisibleiQ13011. HS.

Organism-specific databases

HPAiHPA002907.
HPA005835.

Interactioni

Subunit structurei

Homohexamer.By similarity

Binary interactionsi

WithEntry#Exp.IntActNotes
FAM9BQ8IZU03EBI-711968,EBI-10175124
HTTP428582EBI-711968,EBI-466029
STAT3P407632EBI-711968,EBI-518675
TRAF1Q130773EBI-711968,EBI-359224

GO - Molecular functioni

  • receptor binding Source: UniProtKB

Protein-protein interaction databases

BioGridi108220. 109 interactors.
IntActiQ13011. 40 interactors.
MINTiMINT-1393158.
STRINGi9606.ENSP00000221418.

Structurei

Secondary structure

1328
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi56 – 62Combined sources7
Beta strandi64 – 72Combined sources9
Helixi75 – 77Combined sources3
Helixi83 – 97Combined sources15
Beta strandi104 – 110Combined sources7
Helixi120 – 128Combined sources9
Beta strandi132 – 134Combined sources3
Helixi135 – 158Combined sources24
Beta strandi159 – 161Combined sources3
Beta strandi163 – 167Combined sources5
Beta strandi169 – 172Combined sources4
Helixi174 – 179Combined sources6
Beta strandi182 – 188Combined sources7
Beta strandi192 – 194Combined sources3
Helixi197 – 199Combined sources3
Helixi208 – 215Combined sources8
Helixi219 – 228Combined sources10
Beta strandi231 – 233Combined sources3
Helixi234 – 239Combined sources6
Beta strandi242 – 249Combined sources8
Helixi250 – 265Combined sources16
Helixi269 – 284Combined sources16
Helixi287 – 301Combined sources15
Helixi305 – 313Combined sources9

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2VREX-ray1.95A/B/C50-322[»]
ProteinModelPortaliQ13011.
SMRiQ13011.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ13011.

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni116 – 120Substrate bindingBy similarity5

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi326 – 328Microbody targeting signalSequence analysis3

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG1681. Eukaryota.
ENOG410XTHX. LUCA.
GeneTreeiENSGT00760000119100.
HOGENOMiHOG000027939.
HOVERGENiHBG005556.
InParanoidiQ13011.
KOiK12663.
OMAiSYMRDHR.
OrthoDBiEOG091G0H26.
PhylomeDBiQ13011.
TreeFamiTF314317.

Family and domain databases

Gene3Di1.10.12.10. 1 hit.
3.90.226.10. 1 hit.
InterProiIPR029045. ClpP/crotonase-like_dom.
IPR014748. Crontonase_C.
IPR001753. Crotonase_core_superfam.
IPR018376. Enoyl-CoA_hyd/isom_CS.
[Graphical view]
PfamiPF00378. ECH_1. 1 hit.
[Graphical view]
SUPFAMiSSF52096. SSF52096. 1 hit.
PROSITEiPS00166. ENOYL_COA_HYDRATASE. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

Q13011-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAAGIVASRR LRDLLTRRLT GSNYPGLSIS LRLTGSSAQE EASGVALGEA
60 70 80 90 100
PDHSYESLRV TSAQKHVLHV QLNRPNKRNA MNKVFWREMV ECFNKISRDA
110 120 130 140 150
DCRAVVISGA GKMFTAGIDL MDMASDILQP KGDDVARISW YLRDIITRYQ
160 170 180 190 200
ETFNVIERCP KPVIAAVHGG CIGGGVDLVT ACDIRYCAQD AFFQVKEVDV
210 220 230 240 250
GLAADVGTLQ RLPKVIGNQS LVNELAFTAR KMMADEALGS GLVSRVFPDK
260 270 280 290 300
EVMLDAALAL AAEISSKSPV AVQSTKVNLL YSRDHSVAES LNYVASWNMS
310 320
MLQTQDLVKS VQATTENKEL KTVTFSKL
Length:328
Mass (Da):35,816
Last modified:November 22, 2005 - v2
Checksum:i211E0FF40379E6DA
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti210Q → E in AAC50222 (PubMed:7558027).Curated1
Sequence conflicti210Q → E in AAB86485 (Ref. 2) Curated1
Sequence conflicti230R → H in AAC50222 (PubMed:7558027).Curated1
Sequence conflicti230R → H in AAB86485 (Ref. 2) Curated1
Sequence conflicti239G → D in AAC50222 (PubMed:7558027).Curated1
Sequence conflicti239G → D in AAB86485 (Ref. 2) Curated1
Sequence conflicti259A → P in AAC50222 (PubMed:7558027).Curated1
Sequence conflicti259A → P in AAB86485 (Ref. 2) Curated1
Sequence conflicti262A → P in AAC50222 (PubMed:7558027).Curated1
Sequence conflicti262A → P in AAB86485 (Ref. 2) Curated1
Sequence conflicti268 – 271SPVA → TTVL in AAC50222 (PubMed:7558027).Curated4
Sequence conflicti268 – 271SPVA → TTVL in AAB86485 (Ref. 2) Curated4
Sequence conflicti313A → P in AAC50222 (PubMed:7558027).Curated1
Sequence conflicti313A → P in AAB86485 (Ref. 2) Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_01492741E → A.Combined sources3 PublicationsCorresponds to variant rs9419dbSNPEnsembl.1
Natural variantiVAR_033913217G → R.Corresponds to variant rs2229259dbSNPEnsembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U16660 mRNA. Translation: AAC50222.1.
AF030249
, AF030246, AF030247, AF030248 Genomic DNA. Translation: AAB86485.1.
AK291860 mRNA. Translation: BAF84549.1.
CH471126 Genomic DNA. Translation: EAW56824.1.
BC011792 mRNA. Translation: AAH11792.1.
BC017408 mRNA. Translation: AAH17408.1.
CCDSiCCDS33014.1.
PIRiI38882.
RefSeqiNP_001389.2. NM_001398.2.
UniGeneiHs.196176.

Genome annotation databases

EnsembliENST00000221418; ENSP00000221418; ENSG00000104823.
ENST00000634245; ENSP00000489530; ENSG00000282853.
GeneIDi1891.
KEGGihsa:1891.
UCSCiuc002oji.4. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U16660 mRNA. Translation: AAC50222.1.
AF030249
, AF030246, AF030247, AF030248 Genomic DNA. Translation: AAB86485.1.
AK291860 mRNA. Translation: BAF84549.1.
CH471126 Genomic DNA. Translation: EAW56824.1.
BC011792 mRNA. Translation: AAH11792.1.
BC017408 mRNA. Translation: AAH17408.1.
CCDSiCCDS33014.1.
PIRiI38882.
RefSeqiNP_001389.2. NM_001398.2.
UniGeneiHs.196176.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2VREX-ray1.95A/B/C50-322[»]
ProteinModelPortaliQ13011.
SMRiQ13011.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi108220. 109 interactors.
IntActiQ13011. 40 interactors.
MINTiMINT-1393158.
STRINGi9606.ENSP00000221418.

PTM databases

iPTMnetiQ13011.
PhosphoSitePlusiQ13011.
SwissPalmiQ13011.

Polymorphism and mutation databases

BioMutaiECH1.
DMDMi82654933.

2D gel databases

REPRODUCTION-2DPAGEIPI00011416.
UCD-2DPAGEQ13011.

Proteomic databases

EPDiQ13011.
MaxQBiQ13011.
PaxDbiQ13011.
PeptideAtlasiQ13011.
PRIDEiQ13011.
TopDownProteomicsiQ13011.

Protocols and materials databases

DNASUi1891.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000221418; ENSP00000221418; ENSG00000104823.
ENST00000634245; ENSP00000489530; ENSG00000282853.
GeneIDi1891.
KEGGihsa:1891.
UCSCiuc002oji.4. human.

Organism-specific databases

CTDi1891.
GeneCardsiECH1.
H-InvDBHIX0080117.
HGNCiHGNC:3149. ECH1.
HPAiHPA002907.
HPA005835.
MIMi600696. gene.
neXtProtiNX_Q13011.
OpenTargetsiENSG00000104823.
ENSG00000282853.
PharmGKBiPA27596.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1681. Eukaryota.
ENOG410XTHX. LUCA.
GeneTreeiENSGT00760000119100.
HOGENOMiHOG000027939.
HOVERGENiHBG005556.
InParanoidiQ13011.
KOiK12663.
OMAiSYMRDHR.
OrthoDBiEOG091G0H26.
PhylomeDBiQ13011.
TreeFamiTF314317.

Enzyme and pathway databases

UniPathwayiUPA00659.
BioCyciZFISH:ENSG00000104823-MONOMER.

Miscellaneous databases

ChiTaRSiECH1. human.
EvolutionaryTraceiQ13011.
GeneWikiiECH1.
GenomeRNAii1891.
PROiQ13011.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000104823.
CleanExiHS_ECH1.
ExpressionAtlasiQ13011. baseline and differential.
GenevisibleiQ13011. HS.

Family and domain databases

Gene3Di1.10.12.10. 1 hit.
3.90.226.10. 1 hit.
InterProiIPR029045. ClpP/crotonase-like_dom.
IPR014748. Crontonase_C.
IPR001753. Crotonase_core_superfam.
IPR018376. Enoyl-CoA_hyd/isom_CS.
[Graphical view]
PfamiPF00378. ECH_1. 1 hit.
[Graphical view]
SUPFAMiSSF52096. SSF52096. 1 hit.
PROSITEiPS00166. ENOYL_COA_HYDRATASE. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiECH1_HUMAN
AccessioniPrimary (citable) accession number: Q13011
Secondary accession number(s): A8K745, Q8WVX0, Q96EZ9
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: November 22, 2005
Last modified: November 30, 2016
This is version 166 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 19
    Human chromosome 19: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.