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Protein

Enoyl-CoA delta isomerase 2, mitochondrial

Gene

ECI2

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. Has a preference for 3-trans substrates (By similarity).By similarity

Catalytic activityi

(3Z)-dodec-3-enoyl-CoA = (2E)-dodec-2-enoyl-CoA.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei92Acyl-CoABy similarity1
Binding sitei111Acyl-CoABy similarity1
Sitei280Important for catalytic activityBy similarity1

GO - Molecular functioni

  • dodecenoyl-CoA delta-isomerase activity Source: UniProtKB
  • fatty-acyl-CoA binding Source: InterPro
  • receptor binding Source: UniProtKB

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Isomerase

Enzyme and pathway databases

BioCyciMetaCyc:HS03615-MONOMER.
ZFISH:HS03615-MONOMER.
ReactomeiR-HSA-390918. Peroxisomal lipid metabolism.

Chemistry databases

SwissLipidsiSLP:000001195. [O75521-2]

Names & Taxonomyi

Protein namesi
Recommended name:
Enoyl-CoA delta isomerase 2, mitochondrial (EC:5.3.3.8)
Alternative name(s):
DRS-1
Delta(3),delta(2)-enoyl-CoA isomerase
Short name:
D3,D2-enoyl-CoA isomerase
Diazepam-binding inhibitor-related protein 1
Short name:
DBI-related protein 1
Dodecenoyl-CoA isomerase
Hepatocellular carcinoma-associated antigen 88
Peroxisomal 3,2-trans-enoyl-CoA isomerase
Short name:
pECI
Renal carcinoma antigen NY-REN-1
Gene namesi
Name:ECI2
Synonyms:DRS1, HCA88, PECI
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 6

Organism-specific databases

HGNCiHGNC:14601. ECI2.

Subcellular locationi

Isoform 1 :

GO - Cellular componenti

  • intracellular membrane-bounded organelle Source: HPA
  • membrane Source: UniProtKB
  • mitochondrion Source: LIFEdb
  • nucleoplasm Source: HPA
  • peroxisomal matrix Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion, Peroxisome

Pathology & Biotechi

Organism-specific databases

DisGeNETi10455.
OpenTargetsiENSG00000198721.
PharmGKBiPA33168.

Polymorphism and mutation databases

BioMutaiECI2.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 38MitochondrionSequence analysisAdd BLAST38
ChainiPRO_000021402739 – 394Enoyl-CoA delta isomerase 2, mitochondrialAdd BLAST356

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei51N6-acetyllysine; alternateCombined sources1
Modified residuei51N6-succinyllysine; alternateBy similarity1
Modified residuei55N6-succinyllysineBy similarity1
Modified residuei62N6-acetyllysine; alternateBy similarity1
Modified residuei62N6-succinyllysine; alternateBy similarity1
Modified residuei70N6-succinyllysineBy similarity1
Modified residuei81N6-succinyllysineBy similarity1
Modified residuei90N6-succinyllysineBy similarity1
Modified residuei92N6-acetyllysine; alternateCombined sources1
Modified residuei92N6-succinyllysine; alternateBy similarity1
Modified residuei101PhosphoserineCombined sources1
Modified residuei119PhosphoserineCombined sources1
Modified residuei161N6-succinyllysineBy similarity1
Modified residuei289N6-succinyllysineBy similarity1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiO75521.
MaxQBiO75521.
PaxDbiO75521.
PeptideAtlasiO75521.
PRIDEiO75521.

2D gel databases

REPRODUCTION-2DPAGEIPI00419263.
UCD-2DPAGEO75521.

PTM databases

iPTMnetiO75521.
PhosphoSitePlusiO75521.
SwissPalmiO75521.

Expressioni

Tissue specificityi

Abundant in heart, skeletal muscle and liver. Expressed in CD34+ T-cells and CD34+ bone marrow cells.1 Publication

Gene expression databases

BgeeiENSG00000198721.
ExpressionAtlasiO75521. baseline and differential.
GenevisibleiO75521. HS.

Organism-specific databases

HPAiHPA022130.
HPA031626.

Interactioni

GO - Molecular functioni

  • receptor binding Source: UniProtKB

Protein-protein interaction databases

BioGridi115718. 32 interactors.
IntActiO75521. 12 interactors.
MINTiMINT-3001383.
STRINGi9606.ENSP00000369461.

Structurei

Secondary structure

1394
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi40 – 52Combined sources13
Helixi59 – 69Combined sources11
Turni70 – 74Combined sources5
Helixi87 – 99Combined sources13
Helixi104 – 118Combined sources15
Beta strandi140 – 147Combined sources8
Beta strandi150 – 155Combined sources6
Helixi158 – 160Combined sources3
Helixi166 – 181Combined sources16
Beta strandi185 – 192Combined sources8
Helixi203 – 205Combined sources3
Helixi212 – 232Combined sources21
Beta strandi238 – 242Combined sources5
Helixi249 – 252Combined sources4
Helixi253 – 256Combined sources4
Beta strandi258 – 263Combined sources6
Beta strandi267 – 269Combined sources3
Helixi273 – 275Combined sources3
Helixi283 – 291Combined sources9
Helixi293 – 300Combined sources8
Beta strandi305 – 307Combined sources3
Helixi308 – 313Combined sources6
Beta strandi318 – 321Combined sources4
Turni323 – 325Combined sources3
Helixi326 – 337Combined sources12
Helixi342 – 353Combined sources12
Helixi354 – 356Combined sources3
Helixi357 – 375Combined sources19
Helixi378 – 383Combined sources6
Helixi385 – 388Combined sources4

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2CQUNMR-A31-133[»]
2F6QX-ray1.95A/B/C138-394[»]
4U18X-ray2.64A/B/C138-390[»]
4U19X-ray1.88A/B/C138-390[»]
4U1AX-ray2.85A/B/C138-384[»]
ProteinModelPortaliO75521.
SMRiO75521.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiO75521.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini39 – 124ACBPROSITE-ProRule annotationAdd BLAST86

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni66 – 70Acyl-CoA bindingBy similarity5
Regioni151 – 322ECH-likeAdd BLAST172
Regioni198 – 202Substrate bindingBy similarity5

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi392 – 394Microbody targeting signalSequence analysis3

Sequence similaritiesi

In the C-terminal section; belongs to the enoyl-CoA hydratase/isomerase family.Curated
Contains 1 ACB (acyl-CoA-binding) domain.PROSITE-ProRule annotation

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0016. Eukaryota.
KOG0817. Eukaryota.
COG1024. LUCA.
COG4281. LUCA.
GeneTreeiENSGT00760000119100.
HOVERGENiHBG006723.
InParanoidiO75521.
KOiK13239.
OMAiSDECMNA.
OrthoDBiEOG091G0T5I.
PhylomeDBiO75521.
TreeFamiTF313375.

Family and domain databases

Gene3Di1.20.80.10. 1 hit.
3.90.226.10. 1 hit.
InterProiIPR022408. Acyl-CoA-binding_prot_CS.
IPR000582. Acyl-CoA-binding_protein.
IPR029045. ClpP/crotonase-like_dom.
IPR001753. Crotonase_core_superfam.
IPR014352. FERM/acyl-CoA-bd_prot_3-hlx.
[Graphical view]
PfamiPF00887. ACBP. 1 hit.
PF00378. ECH_1. 1 hit.
[Graphical view]
PRINTSiPR00689. ACOABINDINGP.
SUPFAMiSSF47027. SSF47027. 1 hit.
SSF52096. SSF52096. 1 hit.
PROSITEiPS00880. ACB_1. 1 hit.
PS51228. ACB_2. 1 hit.
[Graphical view]

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: O75521-1) [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
MAMAYLAWRL ARRSCPSSLQ VTSFPVVQLH MNRTAMRASQ KDFENSMNQV
60 70 80 90 100
KLLKKDPGNE VKLKLYALYK QATEGPCNMP KPGVFDLINK AKWDAWNALG
110 120 130 140 150
SLPKEAARQN YVDLVSSLSP SLESSSQVEP GTDRKSTGFE TLVVTSEDGI
160 170 180 190 200
TKIMFNRPKK KNAINTEMYH EIMRALKAAS KDDSIITVLT GNGDYYSSGN
210 220 230 240 250
DLTNFTDIPP GGVEEKAKNN AVLLREFVGC FIDFPKPLIA VVNGPAVGIS
260 270 280 290 300
VTLLGLFDAV YASDRATFHT PFSHLGQSPE GCSSYTFPKI MSPAKATEML
310 320 330 340 350
IFGKKLTAGE ACAQGLVTEV FPDSTFQKEV WTRLKAFAKL PPNALRISKE
360 370 380 390
VIRKREREKL HAVNAEECNV LQGRWLSDEC TNAVVNFLSR KSKL
Length:394
Mass (Da):43,585
Last modified:September 1, 2009 - v4
Checksum:i8AC633D43A320102
GO
Isoform 2 (identifier: O75521-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-35: Missing.

Show »
Length:359
Mass (Da):39,609
Checksum:iB9EC60A0D445C9F0
GO

Sequence cautioni

The sequence AAC19317 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated
The sequence AAD34173 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence AAF66247 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence AAH02668 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated
The sequence AAH16781 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated
The sequence AAH17474 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated
The sequence AAH33841 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated
The sequence AAH34702 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated
The sequence BAG52068 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti119S → C in CAB66577 (PubMed:11230166).Curated1
Sequence conflicti195Y → C in AAC19317 (PubMed:10354522).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_05849347M → I.Corresponds to variant rs3177253dbSNPEnsembl.1
Natural variantiVAR_058494344A → V.2 PublicationsCorresponds to variant rs7166dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0378541 – 35Missing in isoform 2. 3 PublicationsAdd BLAST35

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF069301 mRNA. Translation: AAC19317.1. Different initiation.
AL136642 mRNA. Translation: CAB66577.1.
AK075108 mRNA. Translation: BAG52068.1. Different initiation.
AL033383 Genomic DNA. Translation: CAI42125.1.
AL033383 Genomic DNA. Translation: CAI42127.1.
BC002668 mRNA. Translation: AAH02668.3. Different initiation.
BC016781 mRNA. Translation: AAH16781.1. Different initiation.
BC017474 mRNA. Translation: AAH17474.1. Different initiation.
BC033841 mRNA. Translation: AAH33841.3. Different initiation.
BC034702 mRNA. Translation: AAH34702.1. Different initiation.
AF153612 mRNA. Translation: AAD34173.1. Different initiation.
AF244138 mRNA. Translation: AAF66247.1. Different initiation.
CCDSiCCDS43420.2. [O75521-1]
RefSeqiNP_001159482.1. NM_001166010.1.
NP_006108.2. NM_006117.2.
NP_996667.2. NM_206836.2. [O75521-1]
UniGeneiHs.15250.

Genome annotation databases

EnsembliENST00000380118; ENSP00000369461; ENSG00000198721. [O75521-1]
GeneIDi10455.
KEGGihsa:10455.
UCSCiuc003mwd.4. human. [O75521-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF069301 mRNA. Translation: AAC19317.1. Different initiation.
AL136642 mRNA. Translation: CAB66577.1.
AK075108 mRNA. Translation: BAG52068.1. Different initiation.
AL033383 Genomic DNA. Translation: CAI42125.1.
AL033383 Genomic DNA. Translation: CAI42127.1.
BC002668 mRNA. Translation: AAH02668.3. Different initiation.
BC016781 mRNA. Translation: AAH16781.1. Different initiation.
BC017474 mRNA. Translation: AAH17474.1. Different initiation.
BC033841 mRNA. Translation: AAH33841.3. Different initiation.
BC034702 mRNA. Translation: AAH34702.1. Different initiation.
AF153612 mRNA. Translation: AAD34173.1. Different initiation.
AF244138 mRNA. Translation: AAF66247.1. Different initiation.
CCDSiCCDS43420.2. [O75521-1]
RefSeqiNP_001159482.1. NM_001166010.1.
NP_006108.2. NM_006117.2.
NP_996667.2. NM_206836.2. [O75521-1]
UniGeneiHs.15250.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2CQUNMR-A31-133[»]
2F6QX-ray1.95A/B/C138-394[»]
4U18X-ray2.64A/B/C138-390[»]
4U19X-ray1.88A/B/C138-390[»]
4U1AX-ray2.85A/B/C138-384[»]
ProteinModelPortaliO75521.
SMRiO75521.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi115718. 32 interactors.
IntActiO75521. 12 interactors.
MINTiMINT-3001383.
STRINGi9606.ENSP00000369461.

Chemistry databases

SwissLipidsiSLP:000001195. [O75521-2]

PTM databases

iPTMnetiO75521.
PhosphoSitePlusiO75521.
SwissPalmiO75521.

Polymorphism and mutation databases

BioMutaiECI2.

2D gel databases

REPRODUCTION-2DPAGEIPI00419263.
UCD-2DPAGEO75521.

Proteomic databases

EPDiO75521.
MaxQBiO75521.
PaxDbiO75521.
PeptideAtlasiO75521.
PRIDEiO75521.

Protocols and materials databases

DNASUi10455.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000380118; ENSP00000369461; ENSG00000198721. [O75521-1]
GeneIDi10455.
KEGGihsa:10455.
UCSCiuc003mwd.4. human. [O75521-1]

Organism-specific databases

CTDi10455.
DisGeNETi10455.
GeneCardsiECI2.
H-InvDBHIX0025043.
HGNCiHGNC:14601. ECI2.
HPAiHPA022130.
HPA031626.
MIMi608024. gene.
neXtProtiNX_O75521.
OpenTargetsiENSG00000198721.
PharmGKBiPA33168.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0016. Eukaryota.
KOG0817. Eukaryota.
COG1024. LUCA.
COG4281. LUCA.
GeneTreeiENSGT00760000119100.
HOVERGENiHBG006723.
InParanoidiO75521.
KOiK13239.
OMAiSDECMNA.
OrthoDBiEOG091G0T5I.
PhylomeDBiO75521.
TreeFamiTF313375.

Enzyme and pathway databases

BioCyciMetaCyc:HS03615-MONOMER.
ZFISH:HS03615-MONOMER.
ReactomeiR-HSA-390918. Peroxisomal lipid metabolism.

Miscellaneous databases

EvolutionaryTraceiO75521.
GeneWikiiPECI_(gene).
GenomeRNAii10455.
PROiO75521.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000198721.
ExpressionAtlasiO75521. baseline and differential.
GenevisibleiO75521. HS.

Family and domain databases

Gene3Di1.20.80.10. 1 hit.
3.90.226.10. 1 hit.
InterProiIPR022408. Acyl-CoA-binding_prot_CS.
IPR000582. Acyl-CoA-binding_protein.
IPR029045. ClpP/crotonase-like_dom.
IPR001753. Crotonase_core_superfam.
IPR014352. FERM/acyl-CoA-bd_prot_3-hlx.
[Graphical view]
PfamiPF00887. ACBP. 1 hit.
PF00378. ECH_1. 1 hit.
[Graphical view]
PRINTSiPR00689. ACOABINDINGP.
SUPFAMiSSF47027. SSF47027. 1 hit.
SSF52096. SSF52096. 1 hit.
PROSITEiPS00880. ACB_1. 1 hit.
PS51228. ACB_2. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiECI2_HUMAN
AccessioniPrimary (citable) accession number: O75521
Secondary accession number(s): Q5JYK5
, Q5JYK7, Q7L124, Q8N0X0, Q9BUE9, Q9H0T9, Q9NQH1, Q9NYH7, Q9UN55
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 15, 1999
Last sequence update: September 1, 2009
Last modified: November 30, 2016
This is version 173 of the entry and version 4 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

Caution

It is uncertain whether Met-1 or Met-3 is the initiator.Curated

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 6
    Human chromosome 6: 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. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. 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.