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

Enoyl-CoA delta isomerase 1, mitochondrial

Gene

ECI1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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

(3Z)-dodec-3-enoyl-CoA = (2E)-dodec-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 nitrogenND:1 Publication1
Binding sitei177SubstrateND:1 Publication1
Sitei178Important for catalytic activityIKR:1

GO - Molecular functioni

GO - Biological processi

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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 16

Organism-specific databases

EuPathDBiHostDB:ENSG00000167969.12.
HGNCiHGNC:2703. ECI1.

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 – 41MitochondrionHMP:Add BLAST41
ChainiPRO_000000742042 – 302Enoyl-CoA delta isomerase 1, mitochondrialAdd BLAST261

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei61N6-acetyllysine; alternateHMP:1
Modified residuei61N6-succinyllysine; alternateHMP:1
Modified residuei84N6-succinyllysineHMP:1
Modified residuei89N6-acetyllysineND:1
Modified residuei283N6-acetyllysine; alternateHMP:1
Modified residuei283N6-succinyllysine; alternateHMP:1
Modified residuei288N6-succinyllysineHMP:1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiP42126.
MaxQBiP42126.
PaxDbiP42126.
PeptideAtlasiP42126.
PRIDEiP42126.
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. 9 interactors.
IntActiP42126. 5 interactors.
MINTiMINT-3015421.
STRINGi9606.ENSP00000301729.

Structurei

Secondary structure

1302
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi46 – 52ND:7
Turni53 – 56ND:4
Beta strandi57 – 62ND:6
Turni65 – 67ND:3
Helixi72 – 87ND:16
Beta strandi93 – 100ND:8
Beta strandi102 – 104ND:3
Helixi110 – 113ND:4
Helixi118 – 136ND:19
Beta strandi139 – 146ND:8
Beta strandi148 – 150ND:3
Helixi152 – 158ND:7
Beta strandi161 – 167ND:7
Helixi178 – 181ND:4
Helixi187 – 197ND:11
Helixi199 – 208ND:10
Helixi214 – 219ND:6
Beta strandi222 – 227ND:6
Helixi229 – 231ND:3
Helixi232 – 244ND:13
Helixi248 – 267ND:20
Helixi270 – 281ND:12
Helixi284 – 290ND:7
Helixi293 – 299ND:7

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 bindingND:1 Publication5

Sequence similaritiesi

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.
IPR001753. Crotonase_core_superfam.
IPR018376. Enoyl-CoA_hyd/isom_CS.
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).IKR:1
Sequence conflicti14L → P in AAH19316 (PubMed:15489334).IKR:1
Sequence conflicti41R → P in CAA81065 (PubMed:7829074).IKR:1
Sequence conflicti44S → C AA sequence (PubMed:7818490).IKR:1
Sequence conflicti54G → A in CAA81065 (PubMed:7829074).IKR:1
Sequence conflicti78L → F AA sequence (PubMed:7818490).IKR:1
Sequence conflicti93G → V in AAA35485 (PubMed:8198519).IKR:1
Sequence conflicti114Missing AA sequence (PubMed:7818490).IKR:1
Sequence conflicti192 – 193TL → PY AA sequence (PubMed:7818490).IKR:2
Sequence conflicti204 – 207RALQ → SAPE in AAA35485 (PubMed:8198519).IKR:4
Sequence conflicti210L → S AA sequence (PubMed:7818490).IKR:1
Sequence conflicti213 – 215PPA → RRP in AAA35485 (PubMed:8198519).IKR:3
Sequence conflicti242Q → K AA sequence (PubMed:7818490).IKR:1
Sequence conflicti245A → T AA sequence (PubMed:7818490).IKR:1
Sequence conflicti248D → E AA sequence (PubMed:7818490).IKR:1
Sequence conflicti291Q → E AA sequence (PubMed:7818490).IKR:1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_001358172 – 188Missing in isoform 2. IBD:Add 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: November 22, 2017
This is version 173 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