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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: 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 nitrogenCombined sources1 Publication1
Binding sitei177SubstrateCombined sources1 Publication1
Sitei178Important for catalytic activity1 Publication1

GO - Molecular functioni

Complete GO annotation...

GO - Biological processi

Complete GO annotation...

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

HGNCiHGNC:2703. ECI1.

Subcellular locationi

GO - Cellular componenti

  • extracellular exosome Source: UniProtKB
  • mitochondrial inner membrane Source: Ensembl
  • mitochondrial matrix Source: Reactome
  • mitochondrion Source: UniProtKB
Complete GO annotation...

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.
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 – 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.
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).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

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.

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 7, 2017
This is version 171 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