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Protein

Electron transfer flavoprotein subunit beta

Gene

ETFB

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

Functioni

The electron transfer flavoprotein serves as a specific electron acceptor for several dehydrogenases, including five acyl-CoA dehydrogenases, glutaryl-CoA and sarcosine dehydrogenase. It transfers the electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase).By similarity1 Publication

Cofactori

Protein has several cofactor binding sites:

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei16FAD1 Publication1

GO - Molecular functioni

  • electron carrier activity Source: UniProtKB

GO - Biological processi

Complete GO annotation...

Keywords - Biological processi

Electron transport, Transport

Keywords - Ligandi

FAD, Flavoprotein

Enzyme and pathway databases

BioCyciZFISH:ETFBCLOS-MONOMER.
ReactomeiR-HSA-611105. Respiratory electron transport.

Names & Taxonomyi

Protein namesi
Recommended name:
Electron transfer flavoprotein subunit betaCurated
Short name:
Beta-ETF1 Publication
Gene namesi
Name:ETFBImported
ORF Names:FP585
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 19

Organism-specific databases

HGNCiHGNC:3482. ETFB.

Subcellular locationi

GO - Cellular componenti

  • extracellular exosome Source: UniProtKB
  • intracellular membrane-bounded organelle Source: HPA
  • mitochondrial electron transfer flavoprotein complex Source: GO_Central
  • mitochondrial matrix Source: UniProtKB
  • mitochondrion Source: HPA
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Involvement in diseasei

Glutaric aciduria 2B (GA2B)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn autosomal recessively inherited disorder of fatty acid, amino acid, and choline metabolism. It is characterized by multiple acyl-CoA dehydrogenase deficiencies resulting in large excretion not only of glutaric acid, but also of lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids.
See also OMIM:231680
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_025804128D → N in GA2B. 1 PublicationCorresponds to variant rs104894678dbSNPEnsembl.1
Natural variantiVAR_002369164R → Q in GA2B. 1 PublicationCorresponds to variant rs104894677dbSNPEnsembl.1

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi165E → A or Q: Drastically increases interprotein electron transfer rates. 1 Publication1
Mutagenesisi195L → A: Severely impaired in complex formation with ACADM. 1 Publication1

Keywords - Diseasei

Disease mutation, Glutaricaciduria

Organism-specific databases

DisGeNETi2109.
MalaCardsiETFB.
MIMi231680. phenotype.
OpenTargetsiENSG00000105379.
Orphaneti394532. Multiple acyl-CoA dehydrogenation deficiency, mild type.
394529. Multiple acyl-CoA dehydrogenation deficiency, severe neonatal type.
PharmGKBiPA27898.

Polymorphism and mutation databases

BioMutaiETFB.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedCombined sources
ChainiPRO_00001678702 – 255Electron transfer flavoprotein subunit betaAdd BLAST254

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylalanineCombined sources1
Modified residuei200N6,N6,N6-trimethyllysine; by ETFBKMT; alternate2 Publications1
Modified residuei200N6-acetyllysine; alternateBy similarity1
Modified residuei200N6-methyllysine; alternateCombined sources1
Modified residuei203N6,N6,N6-trimethyllysine; by ETFBKMT2 Publications1
Modified residuei210N6-acetyllysine; alternateBy similarity1
Modified residuei210N6-succinyllysine; alternateBy similarity1
Modified residuei223PhosphoserineCombined sources1
Modified residuei226PhosphoserineCombined sources1
Modified residuei238N6-acetyllysineBy similarity1
Modified residuei248N6-acetyllysine; alternateBy similarity1
Modified residuei248N6-succinyllysine; alternateBy similarity1

Post-translational modificationi

Methylated. Trimethylation at Lys-200 and Lys-203 may negatively regulate the activity in electron transfer from Acyl-CoA dehydrogenases.By similarity2 Publications

Keywords - PTMi

Acetylation, Methylation, Phosphoprotein

Proteomic databases

EPDiP38117.
MaxQBiP38117.
PaxDbiP38117.
PeptideAtlasiP38117.
PRIDEiP38117.
TopDownProteomicsiP38117-1. [P38117-1]
P38117-2. [P38117-2]

2D gel databases

REPRODUCTION-2DPAGEIPI00004902.
UCD-2DPAGEP38117.

PTM databases

iPTMnetiP38117.
PhosphoSitePlusiP38117.
SwissPalmiP38117.

Expressioni

Tissue specificityi

Abundant in liver, heart and skeletal muscle. A weak expression is seen in the brain, placenta, lung, kidney and pancreas.1 Publication

Gene expression databases

BgeeiENSG00000105379.
CleanExiHS_ETFB.
ExpressionAtlasiP38117. baseline and differential.
GenevisibleiP38117. HS.

Organism-specific databases

HPAiHPA018910.
HPA018921.
HPA018923.

Interactioni

Subunit structurei

Electron transfer flavoprotein is a heterodimer composed of ETFA and ETFB (PubMed:8962055). Interacts with ETFRF1 (PubMed:27499296).2 Publications

Protein-protein interaction databases

BioGridi108410. 33 interactors.
DIPiDIP-6162N.
IntActiP38117. 13 interactors.
MINTiMINT-1520912.
STRINGi9606.ENSP00000346173.

Structurei

Secondary structure

1255
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi5 – 9Combined sources5
Beta strandi12 – 14Combined sources3
Beta strandi16 – 18Combined sources3
Beta strandi26 – 29Combined sources4
Beta strandi36 – 38Combined sources3
Helixi40 – 54Combined sources15
Beta strandi59 – 68Combined sources10
Helixi71 – 81Combined sources11
Beta strandi84 – 90Combined sources7
Helixi93 – 96Combined sources4
Helixi101 – 115Combined sources15
Beta strandi118 – 124Combined sources7
Turni127 – 129Combined sources3
Helixi134 – 142Combined sources9
Beta strandi146 – 156Combined sources11
Beta strandi159 – 166Combined sources8
Beta strandi169 – 183Combined sources15
Helixi185 – 187Combined sources3
Helixi195 – 200Combined sources6
Turni201 – 203Combined sources3
Beta strandi206 – 209Combined sources4
Helixi211 – 214Combined sources4
Beta strandi221 – 228Combined sources8
Helixi242 – 251Combined sources10

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1EFVX-ray2.10B1-255[»]
1T9GX-ray2.90S1-255[»]
2A1TX-ray2.80S1-255[»]
2A1UX-ray2.11B1-255[»]
ProteinModelPortaliP38117.
SMRiP38117.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP38117.

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni183 – 205Recognition loop2 PublicationsAdd BLAST23

Domaini

The recognition loop recognizes a hydrophobic patch at the surface of interacting dehydrogenases and acts as a static anchor at the interface.1 Publication

Sequence similaritiesi

Belongs to the ETF beta-subunit/FixA family.Curated

Phylogenomic databases

eggNOGiKOG3180. Eukaryota.
COG2086. LUCA.
GeneTreeiENSGT00390000009936.
HOGENOMiHOG000247877.
HOVERGENiHBG005614.
InParanoidiP38117.
KOiK03521.
OMAiCVISAGP.
OrthoDBiEOG091G0KHH.
PhylomeDBiP38117.
TreeFamiTF314039.

Family and domain databases

CDDicd01714. ETF_beta. 1 hit.
Gene3Di3.40.50.620. 1 hit.
InterProiIPR000049. ET-Flavoprotein_bsu_CS.
IPR014730. ETF_a/b_N.
IPR012255. ETF_b.
IPR033948. ETF_beta_N.
IPR014729. Rossmann-like_a/b/a_fold.
[Graphical view]
PANTHERiPTHR21294. PTHR21294. 1 hit.
PfamiPF01012. ETF. 1 hit.
[Graphical view]
PIRSFiPIRSF000090. Beta-ETF. 1 hit.
SMARTiSM00893. ETF. 1 hit.
[Graphical view]
PROSITEiPS01065. ETF_BETA. 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: P38117-1) [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
MAELRVLVAV KRVIDYAVKI RVKPDRTGVV TDGVKHSMNP FCEIAVEEAV
60 70 80 90 100
RLKEKKLVKE VIAVSCGPAQ CQETIRTALA MGADRGIHVE VPPAEAERLG
110 120 130 140 150
PLQVARVLAK LAEKEKVDLV LLGKQAIDDD CNQTGQMTAG FLDWPQGTFA
160 170 180 190 200
SQVTLEGDKL KVEREIDGGL ETLRLKLPAV VTADLRLNEP RYATLPNIMK
210 220 230 240 250
AKKKKIEVIK PGDLGVDLTS KLSVISVEDP PQRTAGVKVE TTEDLVAKLK

EIGRI
Length:255
Mass (Da):27,844
Last modified:January 23, 2007 - v3
Checksum:i47E6EAEF50EB2C80
GO
Isoform 2 (identifier: P38117-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-19: MAELRVLVAVKRVIDYAVK → MYLSLWVTIN...DPTPSPPAGQ

Show »
Length:346
Mass (Da):37,434
Checksum:i29C992C07350130A
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti198I → S in CAB37832 (PubMed:7912128).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_025804128D → N in GA2B. 1 PublicationCorresponds to variant rs104894678dbSNPEnsembl.1
Natural variantiVAR_008548154T → M.3 PublicationsCorresponds to variant rs1130426dbSNPEnsembl.1
Natural variantiVAR_002369164R → Q in GA2B. 1 PublicationCorresponds to variant rs104894677dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0178501 – 19MAELR…DYAVK → MYLSLWVTINTVNLRNTLSG LRGAVTTVGMIKSDVPGTQE WLDERRRQGDLPLPTNSNPV LSLELCDPGQGPAPFQAVVV LIQPGRGLALRPPPSCLFPP DPTPSPPAGQ in isoform 2. 2 PublicationsAdd BLAST19

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X71129 mRNA. Translation: CAA50441.1.
AF436663
, AF436658, AF436659, AF436660, AF436661, AF436662 Genomic DNA. Translation: AAN03713.1.
AF370381 mRNA. Translation: AAQ15217.1.
CR456827 mRNA. Translation: CAG33108.1.
AK055285 mRNA. Translation: BAG51494.1.
AK291881 mRNA. Translation: BAF84570.1.
BC093961 mRNA. Translation: AAH93961.1.
BC093963 mRNA. Translation: AAH93963.1.
X76067 Genomic DNA. Translation: CAB37832.1.
CCDSiCCDS12828.1. [P38117-1]
CCDS33085.1. [P38117-2]
PIRiS32482.
RefSeqiNP_001014763.1. NM_001014763.1. [P38117-2]
NP_001976.1. NM_001985.2. [P38117-1]
UniGeneiHs.348531.

Genome annotation databases

EnsembliENST00000309244; ENSP00000311930; ENSG00000105379. [P38117-1]
ENST00000354232; ENSP00000346173; ENSG00000105379. [P38117-2]
GeneIDi2109.
KEGGihsa:2109.
UCSCiuc002pwg.4. human. [P38117-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X71129 mRNA. Translation: CAA50441.1.
AF436663
, AF436658, AF436659, AF436660, AF436661, AF436662 Genomic DNA. Translation: AAN03713.1.
AF370381 mRNA. Translation: AAQ15217.1.
CR456827 mRNA. Translation: CAG33108.1.
AK055285 mRNA. Translation: BAG51494.1.
AK291881 mRNA. Translation: BAF84570.1.
BC093961 mRNA. Translation: AAH93961.1.
BC093963 mRNA. Translation: AAH93963.1.
X76067 Genomic DNA. Translation: CAB37832.1.
CCDSiCCDS12828.1. [P38117-1]
CCDS33085.1. [P38117-2]
PIRiS32482.
RefSeqiNP_001014763.1. NM_001014763.1. [P38117-2]
NP_001976.1. NM_001985.2. [P38117-1]
UniGeneiHs.348531.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1EFVX-ray2.10B1-255[»]
1T9GX-ray2.90S1-255[»]
2A1TX-ray2.80S1-255[»]
2A1UX-ray2.11B1-255[»]
ProteinModelPortaliP38117.
SMRiP38117.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi108410. 33 interactors.
DIPiDIP-6162N.
IntActiP38117. 13 interactors.
MINTiMINT-1520912.
STRINGi9606.ENSP00000346173.

PTM databases

iPTMnetiP38117.
PhosphoSitePlusiP38117.
SwissPalmiP38117.

Polymorphism and mutation databases

BioMutaiETFB.

2D gel databases

REPRODUCTION-2DPAGEIPI00004902.
UCD-2DPAGEP38117.

Proteomic databases

EPDiP38117.
MaxQBiP38117.
PaxDbiP38117.
PeptideAtlasiP38117.
PRIDEiP38117.
TopDownProteomicsiP38117-1. [P38117-1]
P38117-2. [P38117-2]

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000309244; ENSP00000311930; ENSG00000105379. [P38117-1]
ENST00000354232; ENSP00000346173; ENSG00000105379. [P38117-2]
GeneIDi2109.
KEGGihsa:2109.
UCSCiuc002pwg.4. human. [P38117-1]

Organism-specific databases

CTDi2109.
DisGeNETi2109.
GeneCardsiETFB.
HGNCiHGNC:3482. ETFB.
HPAiHPA018910.
HPA018921.
HPA018923.
MalaCardsiETFB.
MIMi130410. gene.
231680. phenotype.
neXtProtiNX_P38117.
OpenTargetsiENSG00000105379.
Orphaneti394532. Multiple acyl-CoA dehydrogenation deficiency, mild type.
394529. Multiple acyl-CoA dehydrogenation deficiency, severe neonatal type.
PharmGKBiPA27898.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3180. Eukaryota.
COG2086. LUCA.
GeneTreeiENSGT00390000009936.
HOGENOMiHOG000247877.
HOVERGENiHBG005614.
InParanoidiP38117.
KOiK03521.
OMAiCVISAGP.
OrthoDBiEOG091G0KHH.
PhylomeDBiP38117.
TreeFamiTF314039.

Enzyme and pathway databases

BioCyciZFISH:ETFBCLOS-MONOMER.
ReactomeiR-HSA-611105. Respiratory electron transport.

Miscellaneous databases

EvolutionaryTraceiP38117.
GeneWikiiETFB.
GenomeRNAii2109.
PROiP38117.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000105379.
CleanExiHS_ETFB.
ExpressionAtlasiP38117. baseline and differential.
GenevisibleiP38117. HS.

Family and domain databases

CDDicd01714. ETF_beta. 1 hit.
Gene3Di3.40.50.620. 1 hit.
InterProiIPR000049. ET-Flavoprotein_bsu_CS.
IPR014730. ETF_a/b_N.
IPR012255. ETF_b.
IPR033948. ETF_beta_N.
IPR014729. Rossmann-like_a/b/a_fold.
[Graphical view]
PANTHERiPTHR21294. PTHR21294. 1 hit.
PfamiPF01012. ETF. 1 hit.
[Graphical view]
PIRSFiPIRSF000090. Beta-ETF. 1 hit.
SMARTiSM00893. ETF. 1 hit.
[Graphical view]
PROSITEiPS01065. ETF_BETA. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiETFB_HUMAN
AccessioniPrimary (citable) accession number: P38117
Secondary accession number(s): A8K766
, B3KNY2, Q6IBH7, Q71RF6, Q9Y3S7
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 1, 1994
Last sequence update: January 23, 2007
Last modified: November 30, 2016
This is version 177 of the entry and version 3 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 19
    Human chromosome 19: 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.