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Protein

Enoyl-CoA delta isomerase 2, mitochondrial

Gene

ECI2

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

Caution

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

Catalytic activityi

A (3Z)-alk-3-enoyl-CoA = a (2E)-alk-2-enoyl-CoA.
A (3E)-alk-3-enoyl-CoA = a (2E)-alk-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
  • signaling receptor binding Source: UniProtKB

GO - Biological processi

Keywordsi

Molecular functionIsomerase

Enzyme and pathway databases

BioCyciMetaCyc:HS03615-MONOMER
ReactomeiR-HSA-390247 Beta-oxidation of very long chain fatty acids
R-HSA-9033241 Peroxisomal protein import

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

EuPathDBiHostDB:ENSG00000198721.12
HGNCiHGNC:14601 ECI2
MIMi608024 gene
neXtProtiNX_O75521

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, Peroxisome

Pathology & Biotechi

Organism-specific databases

DisGeNETi10455
OpenTargetsiENSG00000198721
PharmGKBiPA33168

Chemistry databases

DrugBankiDB08231 MYRISTIC ACID

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
ProteomicsDBi50059
50060 [O75521-2]

2D gel databases

REPRODUCTION-2DPAGEiIPI00419263
UCD-2DPAGEiO75521

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

  • signaling receptor binding Source: UniProtKB

Protein-protein interaction databases

BioGridi115718, 37 interactors
IntActiO75521, 53 interactors
MINTiO75521
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

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

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0016 Eukaryota
KOG0817 Eukaryota
COG1024 LUCA
COG4281 LUCA
GeneTreeiENSGT00890000139344
HOVERGENiHBG006723
InParanoidiO75521
KOiK13239
OMAiGCFIDFP
OrthoDBiEOG091G0T5I
PhylomeDBiO75521
TreeFamiTF313375

Family and domain databases

Gene3Di1.10.12.10, 1 hit
1.20.80.10, 1 hit
InterProiView protein in InterPro
IPR022408 Acyl-CoA-binding_prot_CS
IPR000582 Acyl-CoA-binding_protein
IPR035984 Acyl-CoA-binding_sf
IPR029045 ClpP/crotonase-like_dom_sf
IPR001753 Enoyl-CoA_hydra/iso
IPR014748 Enoyl-CoA_hydra_C
IPR014352 FERM/acyl-CoA-bd_prot_sf
PfamiView protein in Pfam
PF00887 ACBP, 1 hit
PF00378 ECH_1, 1 hit
PRINTSiPR00689 ACOABINDINGP
SUPFAMiSSF47027 SSF47027, 1 hit
SSF52096 SSF52096, 1 hit
PROSITEiView protein in PROSITE
PS00880 ACB_1, 1 hit
PS51228 ACB_2, 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: 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 dbSNP:rs3177253Ensembl.1
Natural variantiVAR_058494344A → V2 PublicationsCorresponds to variant dbSNP:rs7166Ensembl.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 No translation available.
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

Similar proteinsi

Entry informationi

Entry nameiECI2_HUMAN
AccessioniPrimary (citable) accession number: O75521
Secondary accession number(s): Q5JYK5
, Q5JYK7, Q7L124, Q8N0X0, Q9BUE9, Q9H0T9, Q9NQH1, Q9NYH7, Q9UN55
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 15, 1999
Last sequence update: September 1, 2009
Last modified: June 20, 2018
This is version 188 of the entry and version 4 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, 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

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