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Protein

2,4-dienoyl-CoA reductase, mitochondrial

Gene

DECR1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Auxiliary enzyme of beta-oxidation. It participates in the metabolism of unsaturated fatty enoyl-CoA esters having double bonds in both even- and odd-numbered positions. Catalyzes the NADP-dependent reduction of 2,4-dienoyl-CoA to yield trans-3-enoyl-CoA.

Catalytic activityi

Trans-2,3-didehydroacyl-CoA + NADP+ = trans,trans-2,3,4,5-tetradehydroacyl-CoA + NADPH.1 Publication

Kineticsi

  1. KM=7.7 µM for NADPH1 Publication
  2. KM=14.3 µM for trans-2,trans-4-hexadienoyl-CoA1 Publication
  1. Vmax=30.3 µmol/min/mg enzyme1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei91NADP1 Publication1
Binding sitei91SubstrateBy similarity1
Binding sitei117NADP1 Publication1
Binding sitei119Substrate1 Publication1
Binding sitei149Substrate1 Publication1
Binding sitei157Substrate1 Publication1
Active sitei199Proton acceptorSequence analysis1
Binding sitei214NADP1 Publication1
Binding sitei251SubstrateBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi66 – 71NADP1 Publication6
Nucleotide bindingi240 – 243NADP1 Publication4

GO - Molecular functioni

  • 2,4-dienoyl-CoA reductase (NADPH) activity Source: UniProtKB
  • NADPH binding Source: UniProtKB
  • oxidoreductase activity, acting on NAD(P)H Source: Reactome

GO - Biological processi

  • fatty acid beta-oxidation Source: UniProtKB
  • protein homotetramerization Source: UniProtKB
Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Biological processi

Fatty acid metabolism, Lipid metabolism

Keywords - Ligandi

NADP

Enzyme and pathway databases

BioCyciZFISH:HS02565-MONOMER.
ReactomeiR-HSA-77288. mitochondrial fatty acid beta-oxidation of unsaturated fatty acids.
SABIO-RKQ16698.

Names & Taxonomyi

Protein namesi
Recommended name:
2,4-dienoyl-CoA reductase, mitochondrial (EC:1.3.1.341 Publication)
Alternative name(s):
2,4-dienoyl-CoA reductase [NADPH]
Short name:
4-enoyl-CoA reductase [NADPH]
Short chain dehydrogenase/reductase family 18C member 1
Gene namesi
Name:DECR1
Synonyms:DECR, SDR18C1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 8

Organism-specific databases

HGNCiHGNC:2753. DECR1.

Subcellular locationi

GO - Cellular componenti

  • cytoplasm Source: HPA
  • extracellular exosome Source: UniProtKB
  • mitochondrial matrix Source: Reactome
  • mitochondrion Source: UniProtKB
  • nucleoplasm Source: HPA
  • nucleus Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Involvement in diseasei

2,4-dienoyl-CoA reductase deficiency (DECRD)1 Publication
The protein represented in this entry is involved in disease pathogenesis. A selective decrease in mitochondrial NADP(H) levels due to NADK2 mutations causes a deficiency of NADPH-dependent mitochondrial enzymes, such as DECR1 and AASS.1 Publication
Disease descriptionA rare, autosomal recessive, inborn error of polyunsaturated fatty acids and lysine metabolism, resulting in mitochondrial dysfunction. Affected individuals have a severe encephalopathy with neurologic and metabolic abnormalities beginning in early infancy. Laboratory studies show increased C10:2 carnitine levels and hyperlysinemia.
See also OMIM:616034

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi148N → A: Reduces enzyme activity by 97%. 1 Publication1
Mutagenesisi199Y → A: Reduces enzyme activity by 99%. Strongly reduced affinity for substrate and for NADP. 1 Publication1
Mutagenesisi210S → A: Reduces enzyme activity by over 99%. 1 Publication1
Mutagenesisi214K → A: Reduces enzyme activity by over 99%. 1 Publication1

Organism-specific databases

DisGeNETi1666.
MalaCardsiDECR1.
MIMi616034. phenotype.
OpenTargetsiENSG00000104325.
PharmGKBiPA141.

Polymorphism and mutation databases

BioMutaiDECR1.
DMDMi3913456.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 34MitochondrionBy similarityAdd BLAST34
ChainiPRO_000003196535 – 3352,4-dienoyl-CoA reductase, mitochondrialAdd BLAST301

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei42N6-acetyllysine; alternateBy similarity1
Modified residuei42N6-succinyllysine; alternateBy similarity1
Modified residuei49N6-acetyllysine; alternateBy similarity1
Modified residuei49N6-succinyllysine; alternateBy similarity1
Modified residuei69PhosphothreonineCombined sources1
Modified residuei73N6-succinyllysineBy similarity1
Modified residuei97N6-acetyllysine; alternateBy similarity1
Modified residuei97N6-succinyllysine; alternateBy similarity1
Modified residuei230N6-acetyllysineCombined sources1
Modified residuei244N6-acetyllysine; alternateBy similarity1
Modified residuei244N6-succinyllysine; alternateBy similarity1
Modified residuei260N6-acetyllysine; alternateBy similarity1
Modified residuei260N6-succinyllysine; alternateBy similarity1
Modified residuei319N6-acetyllysine; alternateBy similarity1
Modified residuei319N6-succinyllysine; alternateBy similarity1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiQ16698.
MaxQBiQ16698.
PaxDbiQ16698.
PeptideAtlasiQ16698.
PRIDEiQ16698.
TopDownProteomicsiQ16698-1. [Q16698-1]
Q16698-2. [Q16698-2]

2D gel databases

UCD-2DPAGEQ16698.

PTM databases

iPTMnetiQ16698.
PhosphoSitePlusiQ16698.
SwissPalmiQ16698.

Expressioni

Tissue specificityi

Heart = liver = pancreas > kidney >> skeletal muscle = lung.1 Publication

Gene expression databases

BgeeiENSG00000104325.
CleanExiHS_DECR1.
ExpressionAtlasiQ16698. baseline and differential.
GenevisibleiQ16698. HS.

Organism-specific databases

HPAiHPA023160.
HPA023162.
HPA023238.

Interactioni

Subunit structurei

Homotetramer.1 Publication

Protein-protein interaction databases

BioGridi108030. 34 interactors.
IntActiQ16698. 22 interactors.
STRINGi9606.ENSP00000220764.

Structurei

Secondary structure

1335
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi36 – 43Combined sources8
Turni54 – 59Combined sources6
Beta strandi61 – 65Combined sources5
Turni66 – 68Combined sources3
Helixi70 – 81Combined sources12
Beta strandi85 – 91Combined sources7
Helixi93 – 107Combined sources15
Beta strandi111 – 115Combined sources5
Helixi121 – 134Combined sources14
Beta strandi139 – 143Combined sources5
Helixi153 – 155Combined sources3
Helixi158 – 185Combined sources28
Beta strandi190 – 195Combined sources6
Helixi199 – 202Combined sources4
Helixi208 – 228Combined sources21
Helixi229 – 231Combined sources3
Beta strandi233 – 240Combined sources8
Beta strandi254 – 256Combined sources3
Helixi257 – 263Combined sources7
Helixi274 – 284Combined sources11
Helixi287 – 289Combined sources3
Beta strandi296 – 300Combined sources5
Helixi303 – 308Combined sources6
Helixi312 – 316Combined sources5
Helixi319 – 325Combined sources7

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1W6UX-ray1.75A/B/C/D35-335[»]
1W73X-ray2.10A/B/C/D35-335[»]
1W8DX-ray2.20A/B/C/D35-335[»]
ProteinModelPortaliQ16698.
SMRiQ16698.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ16698.

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0725. Eukaryota.
COG1028. LUCA.
GeneTreeiENSGT00760000118868.
HOVERGENiHBG005465.
InParanoidiQ16698.
KOiK13236.
OMAiFQSKFFP.
OrthoDBiEOG091G0GO6.
PhylomeDBiQ16698.
TreeFamiTF315256.

Family and domain databases

Gene3Di3.40.50.720. 1 hit.
InterProiIPR016040. NAD(P)-bd_dom.
IPR002347. SDR_fam.
[Graphical view]
PANTHERiPTHR24322. PTHR24322. 2 hits.
PRINTSiPR00081. GDHRDH.
SUPFAMiSSF51735. SSF51735. 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: Q16698-1) [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
MKLPARVFFT LGSRLPCGLA PRRFFSYGTK ILYQNTEALQ SKFFSPLQKA
60 70 80 90 100
MLPPNSFQGK VAFITGGGTG LGKGMTTLLS SLGAQCVIAS RKMDVLKATA
110 120 130 140 150
EQISSQTGNK VHAIQCDVRD PDMVQNTVSE LIKVAGHPNI VINNAAGNFI
160 170 180 190 200
SPTERLSPNA WKTITDIVLN GTAFVTLEIG KQLIKAQKGA AFLSITTIYA
210 220 230 240 250
ETGSGFVVPS ASAKAGVEAM SKSLAAEWGK YGMRFNVIQP GPIKTKGAFS
260 270 280 290 300
RLDPTGTFEK EMIGRIPCGR LGTVEELANL AAFLCSDYAS WINGAVIKFD
310 320 330
GGEEVLISGE FNDLRKVTKE QWDTIEELIR KTKGS
Length:335
Mass (Da):36,068
Last modified:November 1, 1996 - v1
Checksum:iF04E72AACB718430
GO
Isoform 2 (identifier: Q16698-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-23: MKLPARVFFTLGSRLPCGLAPRR → MSGLGKKHLLLMGE

Note: No experimental confirmation available.
Show »
Length:326
Mass (Da):34,994
Checksum:i81B865BEAD0F75A7
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti287D → G in AAB09423 (Ref. 2) Curated1
Sequence conflicti292I → V in AAB09423 (Ref. 2) Curated1
Sequence conflicti303E → G in AAB09423 (Ref. 2) Curated1
Sequence conflicti311F → G in AAB09423 (Ref. 2) Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_012034333K → N.Corresponds to variant rs15094dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0563881 – 23MKLPA…LAPRR → MSGLGKKHLLLMGE in isoform 2. 1 PublicationAdd BLAST23

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
L26050 mRNA. Translation: AAA67551.1.
U49352 mRNA. Translation: AAB09423.1.
U78302
, U94980, U94981, U94982, U94983, U94984, U94985, U94986, U94987 Genomic DNA. Translation: AAB88724.1.
AK300069 mRNA. Translation: BAH13204.1.
AC004612 Genomic DNA. Translation: AAC14671.1.
AF049895 Genomic DNA. No translation available.
BC105080 mRNA. Translation: AAI05081.1.
BC105082 mRNA. Translation: AAI05083.1.
CCDSiCCDS6250.1. [Q16698-1]
PIRiS53352.
RefSeqiNP_001317504.1. NM_001330575.1.
NP_001350.1. NM_001359.1. [Q16698-1]
XP_016868636.1. XM_017013147.1. [Q16698-2]
XP_016868637.1. XM_017013148.1. [Q16698-2]
UniGeneiHs.492212.
Hs.660770.

Genome annotation databases

EnsembliENST00000220764; ENSP00000220764; ENSG00000104325. [Q16698-1]
ENST00000522161; ENSP00000429779; ENSG00000104325. [Q16698-2]
GeneIDi1666.
KEGGihsa:1666.
UCSCiuc003yek.2. human. [Q16698-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
L26050 mRNA. Translation: AAA67551.1.
U49352 mRNA. Translation: AAB09423.1.
U78302
, U94980, U94981, U94982, U94983, U94984, U94985, U94986, U94987 Genomic DNA. Translation: AAB88724.1.
AK300069 mRNA. Translation: BAH13204.1.
AC004612 Genomic DNA. Translation: AAC14671.1.
AF049895 Genomic DNA. No translation available.
BC105080 mRNA. Translation: AAI05081.1.
BC105082 mRNA. Translation: AAI05083.1.
CCDSiCCDS6250.1. [Q16698-1]
PIRiS53352.
RefSeqiNP_001317504.1. NM_001330575.1.
NP_001350.1. NM_001359.1. [Q16698-1]
XP_016868636.1. XM_017013147.1. [Q16698-2]
XP_016868637.1. XM_017013148.1. [Q16698-2]
UniGeneiHs.492212.
Hs.660770.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1W6UX-ray1.75A/B/C/D35-335[»]
1W73X-ray2.10A/B/C/D35-335[»]
1W8DX-ray2.20A/B/C/D35-335[»]
ProteinModelPortaliQ16698.
SMRiQ16698.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi108030. 34 interactors.
IntActiQ16698. 22 interactors.
STRINGi9606.ENSP00000220764.

PTM databases

iPTMnetiQ16698.
PhosphoSitePlusiQ16698.
SwissPalmiQ16698.

Polymorphism and mutation databases

BioMutaiDECR1.
DMDMi3913456.

2D gel databases

UCD-2DPAGEQ16698.

Proteomic databases

EPDiQ16698.
MaxQBiQ16698.
PaxDbiQ16698.
PeptideAtlasiQ16698.
PRIDEiQ16698.
TopDownProteomicsiQ16698-1. [Q16698-1]
Q16698-2. [Q16698-2]

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000220764; ENSP00000220764; ENSG00000104325. [Q16698-1]
ENST00000522161; ENSP00000429779; ENSG00000104325. [Q16698-2]
GeneIDi1666.
KEGGihsa:1666.
UCSCiuc003yek.2. human. [Q16698-1]

Organism-specific databases

CTDi1666.
DisGeNETi1666.
GeneCardsiDECR1.
HGNCiHGNC:2753. DECR1.
HPAiHPA023160.
HPA023162.
HPA023238.
MalaCardsiDECR1.
MIMi222745. gene.
616034. phenotype.
neXtProtiNX_Q16698.
OpenTargetsiENSG00000104325.
PharmGKBiPA141.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0725. Eukaryota.
COG1028. LUCA.
GeneTreeiENSGT00760000118868.
HOVERGENiHBG005465.
InParanoidiQ16698.
KOiK13236.
OMAiFQSKFFP.
OrthoDBiEOG091G0GO6.
PhylomeDBiQ16698.
TreeFamiTF315256.

Enzyme and pathway databases

BioCyciZFISH:HS02565-MONOMER.
ReactomeiR-HSA-77288. mitochondrial fatty acid beta-oxidation of unsaturated fatty acids.
SABIO-RKQ16698.

Miscellaneous databases

ChiTaRSiDECR1. human.
EvolutionaryTraceiQ16698.
GenomeRNAii1666.
PROiQ16698.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000104325.
CleanExiHS_DECR1.
ExpressionAtlasiQ16698. baseline and differential.
GenevisibleiQ16698. HS.

Family and domain databases

Gene3Di3.40.50.720. 1 hit.
InterProiIPR016040. NAD(P)-bd_dom.
IPR002347. SDR_fam.
[Graphical view]
PANTHERiPTHR24322. PTHR24322. 2 hits.
PRINTSiPR00081. GDHRDH.
SUPFAMiSSF51735. SSF51735. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiDECR_HUMAN
AccessioniPrimary (citable) accession number: Q16698
Secondary accession number(s): B7Z6B8, Q2M304, Q93085
Entry historyi
Integrated into UniProtKB/Swiss-Prot: December 15, 1998
Last sequence update: November 1, 1996
Last modified: November 30, 2016
This is version 155 of the entry and version 1 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

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. Human chromosome 8
    Human chromosome 8: 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.