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UniProtKB - P42126 (ECI1_HUMAN)

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Protein

Enoyl-CoA delta isomerase 1, mitochondrial

Gene

ECI1

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

Functioni

Able to isomerize both 3-cis and 3-trans double bonds into the 2-trans form in a range of enoyl-CoA species.

Catalytic activityi

A (3Z)-alk-3-enoyl-CoA = a (2E)-alk-2-enoyl-CoA.1 Publication
A (3E)-alk-3-enoyl-CoA = a (2E)-alk-2-enoyl-CoA.1 Publication

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 sitei153Substrate; via amide nitrogenCombined sources1 Publication1
Binding sitei177SubstrateCombined sources1 Publication1
Sitei178Important for catalytic activity1 Publication1

GO - Molecular functioni

View the complete GO annotation on QuickGO ...

GO - Biological processi

View the complete GO annotation on QuickGO ...

Keywordsi

Molecular functionIsomerase
Biological processFatty acid metabolism, Lipid metabolism

Enzyme and pathway databases

BRENDAi5.3.3.8 2681
ReactomeiR-HSA-77288 mitochondrial fatty acid beta-oxidation of unsaturated fatty acids
UniPathwayiUPA00659

Chemistry databases

SwissLipidsiSLP:000001184

Names & Taxonomyi

Protein namesi
Recommended name:
Enoyl-CoA delta isomerase 1, mitochondrial (EC:5.3.3.8)
Alternative name(s):
3,2-trans-enoyl-CoA isomerase
Delta(3),Delta(2)-enoyl-CoA isomerase
Short name:
D3,D2-enoyl-CoA isomerase
Dodecenoyl-CoA isomerase
Gene namesi
Name:ECI1
Synonyms:DCI
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo
Proteomesi
  • UP000005640 Componenti: Chromosome 16

Organism-specific databases

EuPathDBiHostDB:ENSG00000167969.12
HGNCiHGNC:2703 ECI1
MIMi600305 gene
neXtProtiNX_P42126

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

Mitochondrion

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi178E → A: Loss of activity. 1 Publication1

Organism-specific databases

DisGeNETi1632
OpenTargetsiENSG00000167969
PharmGKBiPA27173

Chemistry databases

DrugBankiDB03127 Benzamidine
DB02910 Octanoyl-Coenzyme A

Polymorphism and mutation databases

BioMutaiECI1
DMDMi1169204

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 41MitochondrionBy similarityAdd BLAST41
ChainiPRO_000000742042 – 302Enoyl-CoA delta isomerase 1, mitochondrialAdd BLAST261

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei61N6-acetyllysine; alternateBy similarity1
Modified residuei61N6-succinyllysine; alternateBy similarity1
Modified residuei84N6-succinyllysineBy similarity1
Modified residuei89N6-acetyllysineCombined sources1
Modified residuei283N6-acetyllysine; alternateBy similarity1
Modified residuei283N6-succinyllysine; alternateBy similarity1
Modified residuei288N6-succinyllysineBy similarity1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiP42126
MaxQBiP42126
PaxDbiP42126
PeptideAtlasiP42126
PRIDEiP42126
ProteomicsDBi55485
55486 [P42126-2]
TopDownProteomicsiP42126-1 [P42126-1]
P42126-2 [P42126-2]

2D gel databases

OGPiP42126

PTM databases

iPTMnetiP42126
PhosphoSitePlusiP42126
SwissPalmiP42126

Expressioni

Gene expression databases

BgeeiENSG00000167969
CleanExiHS_DCI
ExpressionAtlasiP42126 baseline and differential
GenevisibleiP42126 HS

Organism-specific databases

HPAiHPA041746
HPA043227

Interactioni

Subunit structurei

Homotrimer.1 Publication

Protein-protein interaction databases

BioGridi108000, 10 interactors
IntActiP42126, 7 interactors
MINTiP42126
STRINGi9606.ENSP00000301729

Structurei

Secondary structure

1302
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi46 – 52Combined sources7
Turni53 – 56Combined sources4
Beta strandi57 – 62Combined sources6
Turni65 – 67Combined sources3
Helixi72 – 87Combined sources16
Beta strandi93 – 100Combined sources8
Beta strandi102 – 104Combined sources3
Helixi110 – 113Combined sources4
Helixi118 – 136Combined sources19
Beta strandi139 – 146Combined sources8
Beta strandi148 – 150Combined sources3
Helixi152 – 158Combined sources7
Beta strandi161 – 167Combined sources7
Helixi178 – 181Combined sources4
Helixi187 – 197Combined sources11
Helixi199 – 208Combined sources10
Helixi214 – 219Combined sources6
Beta strandi222 – 227Combined sources6
Helixi229 – 231Combined sources3
Helixi232 – 244Combined sources13
Helixi248 – 267Combined sources20
Helixi270 – 281Combined sources12
Helixi284 – 290Combined sources7
Helixi293 – 299Combined sources7

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1SG4X-ray1.30A/B/C43-302[»]
ProteinModelPortaliP42126
SMRiP42126
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP42126

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni106 – 110Substrate bindingCombined sources1 Publication5

Sequence similaritiesi

Belongs to the enoyl-CoA hydratase/isomerase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiENOG410ISNQ Eukaryota
COG1024 LUCA
GeneTreeiENSGT00390000005678
HOVERGENiHBG001112
InParanoidiP42126
KOiK13238
OMAiTMHHAGI
OrthoDBiEOG091G0I32
PhylomeDBiP42126
TreeFamiTF314436

Family and domain databases

InterProiView protein in InterPro
IPR029045 ClpP/crotonase-like_dom_sf
IPR018376 Enoyl-CoA_hyd/isom_CS
IPR001753 Enoyl-CoA_hydra/iso
PfamiView protein in Pfam
PF00378 ECH_1, 1 hit
SUPFAMiSSF52096 SSF52096, 1 hit
PROSITEiView protein in PROSITE
PS00166 ENOYL_COA_HYDRATASE, 1 hit

Sequences (2)i

Sequence statusi: Complete.

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

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: P42126-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MALVASVRVP ARVLLRAGAR LPGAALGRTE RAAGGGDGAR RFGSQRVLVE 
        60         70         80         90        100
PDAGAGVAVM KFKNPPVNSL SLEFLTELVI SLEKLENDKS FRGVILTSDR 
       110        120        130        140        150
PGVFSAGLDL TEMCGRSPAH YAGYWKAVQE LWLRLYQSNL VLVSAINGAC 
       160        170        180        190        200
PAGGCLVALT CDYRILADNP RYCIGLNETQ LGIIAPFWLK DTLENTIGHR 
       210        220        230        240        250
AAERALQLGL LFPPAEALQV GIVDQVVPEE QVQSTALSAI AQWMAIPDHA 
       260        270        280        290        300
RQLTKAMMRK ATASRLVTQR DADVQNFVSF ISKDSIQKSL QMYLERLKEE 

KG
Length:302
Mass (Da):32,816
Last modified:November 1, 1995 - v1
Checksum:i6E63827DE9BE886B
GO
Isoform 2 (identifier: P42126-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     172-188: Missing.

Note: No experimental confirmation available.
Show »
Length:285
Mass (Da):30,896
Checksum:iF3689BA4B6F7FC72
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti14L → P in AAH00762 (PubMed:15489334).Curated1
Sequence conflicti14L → P in AAH19316 (PubMed:15489334).Curated1
Sequence conflicti41R → P in CAA81065 (PubMed:7829074).Curated1
Sequence conflicti44S → C AA sequence (PubMed:7818490).Curated1
Sequence conflicti54G → A in CAA81065 (PubMed:7829074).Curated1
Sequence conflicti78L → F AA sequence (PubMed:7818490).Curated1
Sequence conflicti93G → V in AAA35485 (PubMed:8198519).Curated1
Sequence conflicti114Missing AA sequence (PubMed:7818490).Curated1
Sequence conflicti192 – 193TL → PY AA sequence (PubMed:7818490).Curated2
Sequence conflicti204 – 207RALQ → SAPE in AAA35485 (PubMed:8198519).Curated4
Sequence conflicti210L → S AA sequence (PubMed:7818490).Curated1
Sequence conflicti213 – 215PPA → RRP in AAA35485 (PubMed:8198519).Curated3
Sequence conflicti242Q → K AA sequence (PubMed:7818490).Curated1
Sequence conflicti245A → T AA sequence (PubMed:7818490).Curated1
Sequence conflicti248D → E AA sequence (PubMed:7818490).Curated1
Sequence conflicti291Q → E AA sequence (PubMed:7818490).Curated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_001358172 – 188Missing in isoform 2. 1 PublicationAdd BLAST17

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Z25820 mRNA Translation: CAA81065.1
Z25821
, Z25822, Z25823, Z25824 Genomic DNA Translation: CAA81066.1
AK291127 mRNA Translation: BAF83816.1
CH471112 Genomic DNA Translation: EAW85524.1
BC000762 mRNA Translation: AAH00762.1
BC002746 mRNA Translation: AAH02746.1
BC020228 mRNA Translation: AAH20228.1
BC019316 mRNA Translation: AAH19316.1
L24774 mRNA Translation: AAA35485.1
CCDSiCCDS10464.1 [P42126-1]
CCDS58410.1 [P42126-2]
PIRiA55723
RefSeqiNP_001171500.1, NM_001178029.1 [P42126-2]
NP_001910.2, NM_001919.3 [P42126-1]
UniGeneiHs.403436

Genome annotation databases

EnsembliENST00000301729; ENSP00000301729; ENSG00000167969 [P42126-1]
ENST00000562238; ENSP00000456319; ENSG00000167969 [P42126-2]
GeneIDi1632
KEGGihsa:1632
UCSCiuc002cpr.4 human [P42126-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiECI1_HUMAN
AccessioniPrimary (citable) accession number: P42126
Secondary accession number(s): A8K512
, Q13290, Q7Z2L6, Q7Z2L7, Q9BUB8, Q9BW05, Q9UDG6
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1995
Last sequence update: November 1, 1995
Last modified: June 20, 2018
This is version 178 of the entry and version 1 of the sequence. See 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 16
    Human chromosome 16: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  4. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  5. SIMILARITY comments
    Index of protein domains and families

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