UniProtKB - P42126 (ECI1_HUMAN)
Protein
Enoyl-CoA delta isomerase 1, mitochondrial
Gene
ECI1
Organism
Homo sapiens (Human)
Status
Functioni
Able to isomerize both 3-cis and 3-trans double bonds into the 2-trans form in a range of enoyl-CoA species.1 Publication
Catalytic activityi
- EC:5.3.3.81 PublicationThis reaction proceeds in the forward1 Publication direction.
- EC:5.3.3.81 PublicationThis reaction proceeds in the forward1 Publication direction.
- This reaction proceeds in the forward1 Publication direction.
- This reaction proceeds in the backwardBy similarity direction.
: fatty acid beta-oxidation Pathwayi
This protein is involved in the pathway fatty acid beta-oxidation, which is part of Lipid metabolism.1 PublicationView all proteins of this organism that are known to be involved in the pathway fatty acid beta-oxidation and in Lipid metabolism.
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Binding sitei | 153 | Substrate; via amide nitrogenCombined sources1 Publication | 1 | |
Binding sitei | 177 | SubstrateCombined sources1 Publication | 1 | |
Sitei | 178 | Important for catalytic activity1 Publication | 1 |
GO - Molecular functioni
- dodecenoyl-CoA delta-isomerase activity Source: UniProtKB
- enoyl-CoA hydratase activity Source: GO_Central
- intramolecular oxidoreductase activity, transposing C=C bonds Source: UniProtKB
GO - Biological processi
- fatty acid beta-oxidation Source: UniProtKB
Keywordsi
Molecular function | Isomerase |
Biological process | Fatty acid metabolism, Lipid metabolism |
Enzyme and pathway databases
BRENDAi | 5.3.3.8, 2681 |
PathwayCommonsi | P42126 |
Reactomei | R-HSA-77288, mitochondrial fatty acid beta-oxidation of unsaturated fatty acids |
UniPathwayi | UPA00659 |
Chemistry databases
SwissLipidsi | SLP: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 |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
EuPathDBi | HostDB:ENSG00000167969.12 |
HGNCi | HGNC:2703, ECI1 |
MIMi | 600305, gene |
neXtProti | NX_P42126 |
Subcellular locationi
Mitochondrion
- Mitochondrion matrix By similarity
Mitochondrion
- mitochondrial matrix Source: Reactome
- mitochondrion Source: HPA
Keywords - Cellular componenti
MitochondrionPathology & Biotechi
Mutagenesis
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Mutagenesisi | 178 | E → A: Loss of activity. 1 Publication | 1 |
Organism-specific databases
DisGeNETi | 1632 |
OpenTargetsi | ENSG00000167969 |
PharmGKBi | PA27173 |
Miscellaneous databases
Pharosi | P42126, Tbio |
Chemistry databases
DrugBanki | DB03127, Benzamidine DB02910, Octanoyl-Coenzyme A |
Polymorphism and mutation databases
BioMutai | ECI1 |
DMDMi | 1169204 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Transit peptidei | 1 – 41 | MitochondrionBy similarityAdd BLAST | 41 | |
ChainiPRO_0000007420 | 42 – 302 | Enoyl-CoA delta isomerase 1, mitochondrialAdd BLAST | 261 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 61 | N6-acetyllysine; alternateBy similarity | 1 | |
Modified residuei | 61 | N6-succinyllysine; alternateBy similarity | 1 | |
Modified residuei | 84 | N6-succinyllysineBy similarity | 1 | |
Modified residuei | 89 | N6-acetyllysineCombined sources | 1 | |
Modified residuei | 283 | N6-acetyllysine; alternateBy similarity | 1 | |
Modified residuei | 283 | N6-succinyllysine; alternateBy similarity | 1 | |
Modified residuei | 288 | N6-succinyllysineBy similarity | 1 |
Keywords - PTMi
AcetylationProteomic databases
EPDi | P42126 |
jPOSTi | P42126 |
MassIVEi | P42126 |
MaxQBi | P42126 |
PaxDbi | P42126 |
PeptideAtlasi | P42126 |
PRIDEi | P42126 |
ProteomicsDBi | 55485 [P42126-1] 55486 [P42126-2] |
TopDownProteomicsi | P42126-1 [P42126-1] P42126-2 [P42126-2] |
2D gel databases
OGPi | P42126 |
PTM databases
iPTMneti | P42126 |
PhosphoSitePlusi | P42126 |
SwissPalmi | P42126 |
Expressioni
Tissue specificityi
Expressed in liver (at protein level).1 Publication
Gene expression databases
Bgeei | ENSG00000167969, Expressed in apex of heart and 230 other tissues |
ExpressionAtlasi | P42126, baseline and differential |
Genevisiblei | P42126, HS |
Organism-specific databases
HPAi | ENSG00000167969, Tissue enhanced (skeletal) |
Interactioni
Subunit structurei
Homotrimer.
1 PublicationProtein-protein interaction databases
BioGRIDi | 108000, 40 interactors |
IntActi | P42126, 9 interactors |
MINTi | P42126 |
STRINGi | 9606.ENSP00000301729 |
Miscellaneous databases
RNActi | P42126, protein |
Structurei
Secondary structure
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details3D structure databases
SMRi | P42126 |
ModBasei | Search... |
PDBe-KBi | Search... |
Miscellaneous databases
EvolutionaryTracei | P42126 |
Family & Domainsi
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 106 – 110 | Substrate bindingCombined sources1 Publication | 5 |
Sequence similaritiesi
Belongs to the enoyl-CoA hydratase/isomerase family.Curated
Keywords - Domaini
Transit peptidePhylogenomic databases
eggNOGi | KOG1683, Eukaryota |
GeneTreei | ENSGT00390000005678 |
InParanoidi | P42126 |
OMAi | TMHHAGI |
OrthoDBi | 974911at2759 |
PhylomeDBi | P42126 |
TreeFami | TF314436 |
Family and domain databases
InterProi | View protein in InterPro IPR029045, ClpP/crotonase-like_dom_sf IPR018376, Enoyl-CoA_hyd/isom_CS IPR001753, Enoyl-CoA_hydra/iso |
Pfami | View protein in Pfam PF00378, ECH_1, 1 hit |
SUPFAMi | SSF52096, SSF52096, 1 hit |
PROSITEi | View protein in PROSITE PS00166, ENOYL_COA_HYDRATASE, 1 hit |
s (2+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry describes 2 produced by isoformsialternative splicing. AlignAdd to basketThis entry has 2 described isoforms and 2 potential isoforms that are computationally mapped.Show allAlign All
Isoform 1 (identifier: P42126-1) [UniParc]FASTAAdd to basket
This isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
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
Computationally mapped potential isoform sequencesi
There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketQ96DC0 | Q96DC0_HUMAN | DCI protein | ECI1 DCI, hCG_41028 | 243 | Annotation score: | ||
H3BS70 | H3BS70_HUMAN | Enoyl-CoA delta isomerase 1, mitoch... | ECI1 | 224 | Annotation score: |
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 14 | L → P in AAH00762 (PubMed:15489334).Curated | 1 | |
Sequence conflicti | 14 | L → P in AAH19316 (PubMed:15489334).Curated | 1 | |
Sequence conflicti | 41 | R → P in CAA81065 (PubMed:7829074).Curated | 1 | |
Sequence conflicti | 44 | S → C AA sequence (PubMed:7818490).Curated | 1 | |
Sequence conflicti | 54 | G → A in CAA81065 (PubMed:7829074).Curated | 1 | |
Sequence conflicti | 78 | L → F AA sequence (PubMed:7818490).Curated | 1 | |
Sequence conflicti | 93 | G → V in AAA35485 (PubMed:8198519).Curated | 1 | |
Sequence conflicti | 114 | Missing AA sequence (PubMed:7818490).Curated | 1 | |
Sequence conflicti | 192 – 193 | TL → PY AA sequence (PubMed:7818490).Curated | 2 | |
Sequence conflicti | 204 – 207 | RALQ → SAPE in AAA35485 (PubMed:8198519).Curated | 4 | |
Sequence conflicti | 210 | L → S AA sequence (PubMed:7818490).Curated | 1 | |
Sequence conflicti | 213 – 215 | PPA → RRP in AAA35485 (PubMed:8198519).Curated | 3 | |
Sequence conflicti | 242 | Q → K AA sequence (PubMed:7818490).Curated | 1 | |
Sequence conflicti | 245 | A → T AA sequence (PubMed:7818490).Curated | 1 | |
Sequence conflicti | 248 | D → E AA sequence (PubMed:7818490).Curated | 1 | |
Sequence conflicti | 291 | Q → E AA sequence (PubMed:7818490).Curated | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_001358 | 172 – 188 | Missing in isoform 2. 1 PublicationAdd BLAST | 17 |
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 |
CCDSi | CCDS10464.1 [P42126-1] CCDS58410.1 [P42126-2] |
PIRi | A55723 |
RefSeqi | NP_001171500.1, NM_001178029.1 [P42126-2] NP_001910.2, NM_001919.3 [P42126-1] |
Genome annotation databases
Ensembli | ENST00000301729; ENSP00000301729; ENSG00000167969 [P42126-1] ENST00000562238; ENSP00000456319; ENSG00000167969 [P42126-2] |
GeneIDi | 1632 |
KEGGi | hsa:1632 |
UCSCi | uc002cpr.4, human [P42126-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
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 |
CCDSi | CCDS10464.1 [P42126-1] CCDS58410.1 [P42126-2] |
PIRi | A55723 |
RefSeqi | NP_001171500.1, NM_001178029.1 [P42126-2] NP_001910.2, NM_001919.3 [P42126-1] |
3D structure databases
Select the link destinations: PDBei RCSB PDBi PDBji Links Updated | PDB entry | Method | Resolution (Å) | Chain | Positions | PDBsum |
1SG4 | X-ray | 1.30 | A/B/C | 43-302 | [»] | |
SMRi | P42126 | |||||
ModBasei | Search... | |||||
PDBe-KBi | Search... |
Protein-protein interaction databases
BioGRIDi | 108000, 40 interactors |
IntActi | P42126, 9 interactors |
MINTi | P42126 |
STRINGi | 9606.ENSP00000301729 |
Chemistry databases
DrugBanki | DB03127, Benzamidine DB02910, Octanoyl-Coenzyme A |
SwissLipidsi | SLP:000001184 |
PTM databases
iPTMneti | P42126 |
PhosphoSitePlusi | P42126 |
SwissPalmi | P42126 |
Polymorphism and mutation databases
BioMutai | ECI1 |
DMDMi | 1169204 |
2D gel databases
OGPi | P42126 |
Proteomic databases
EPDi | P42126 |
jPOSTi | P42126 |
MassIVEi | P42126 |
MaxQBi | P42126 |
PaxDbi | P42126 |
PeptideAtlasi | P42126 |
PRIDEi | P42126 |
ProteomicsDBi | 55485 [P42126-1] 55486 [P42126-2] |
TopDownProteomicsi | P42126-1 [P42126-1] P42126-2 [P42126-2] |
Protocols and materials databases
Antibodypediai | 23677, 272 antibodies |
Genome annotation databases
Ensembli | ENST00000301729; ENSP00000301729; ENSG00000167969 [P42126-1] ENST00000562238; ENSP00000456319; ENSG00000167969 [P42126-2] |
GeneIDi | 1632 |
KEGGi | hsa:1632 |
UCSCi | uc002cpr.4, human [P42126-1] |
Organism-specific databases
CTDi | 1632 |
DisGeNETi | 1632 |
EuPathDBi | HostDB:ENSG00000167969.12 |
GeneCardsi | ECI1 |
HGNCi | HGNC:2703, ECI1 |
HPAi | ENSG00000167969, Tissue enhanced (skeletal) |
MIMi | 600305, gene |
neXtProti | NX_P42126 |
OpenTargetsi | ENSG00000167969 |
PharmGKBi | PA27173 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG1683, Eukaryota |
GeneTreei | ENSGT00390000005678 |
InParanoidi | P42126 |
OMAi | TMHHAGI |
OrthoDBi | 974911at2759 |
PhylomeDBi | P42126 |
TreeFami | TF314436 |
Enzyme and pathway databases
UniPathwayi | UPA00659 |
BRENDAi | 5.3.3.8, 2681 |
PathwayCommonsi | P42126 |
Reactomei | R-HSA-77288, mitochondrial fatty acid beta-oxidation of unsaturated fatty acids |
Miscellaneous databases
BioGRID-ORCSi | 1632, 5 hits in 840 CRISPR screens |
ChiTaRSi | ECI1, human |
EvolutionaryTracei | P42126 |
GenomeRNAii | 1632 |
Pharosi | P42126, Tbio |
PROi | PR:P42126 |
RNActi | P42126, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000167969, Expressed in apex of heart and 230 other tissues |
ExpressionAtlasi | P42126, baseline and differential |
Genevisiblei | P42126, HS |
Family and domain databases
InterProi | View protein in InterPro IPR029045, ClpP/crotonase-like_dom_sf IPR018376, Enoyl-CoA_hyd/isom_CS IPR001753, Enoyl-CoA_hydra/iso |
Pfami | View protein in Pfam PF00378, ECH_1, 1 hit |
SUPFAMi | SSF52096, SSF52096, 1 hit |
PROSITEi | View protein in PROSITE PS00166, ENOYL_COA_HYDRATASE, 1 hit |
ProtoNeti | Search... |
MobiDBi | Search... |
Entry informationi
Entry namei | ECI1_HUMAN | |
Accessioni | P42126Primary (citable) accession number: P42126 Secondary accession number(s): A8K512 Q9UDG6 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | November 1, 1995 |
Last sequence update: | November 1, 1995 | |
Last modified: | December 2, 2020 | |
This is version 194 of the entry and version 1 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any 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, Direct protein sequencing, Reference proteomeDocuments
- MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PATHWAY comments
Index of metabolic and biosynthesis pathways - PDB cross-references
Index of Protein Data Bank (PDB) cross-references - SIMILARITY comments
Index of protein domains and families - Human chromosome 16
Human chromosome 16: entries, gene names and cross-references to MIM