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Protein

Electron transfer flavoprotein subunit beta

Gene

ETFB

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

Functioni

Heterodimeric electron transfer flavoprotein that accepts electrons from several mitochondrial dehydrogenases, including acyl-CoA dehydrogenases, glutaryl-CoA and sarcosine dehydrogenase (PubMed:25416781, PubMed:15159392, PubMed:15975918). It transfers the electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (Probable). Required for normal mitochondrial fatty acid oxidation and normal amino acid metabolism (PubMed:12815589, PubMed:7912128). ETFB binds an AMP molecule that probably has a purely structural role (PubMed:8962055, PubMed:15159392, PubMed:15975918).1 PublicationCurated6 Publications

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei9AMP; via carbonyl oxygenCombined sources3 Publications1
Binding sitei66AMP; via amide nitrogen and carbonyl oxygenCombined sources3 Publications1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi39 – 42AMPCombined sources3 Publications4
Nucleotide bindingi123 – 134AMPCombined sources3 PublicationsAdd BLAST12

GO - Molecular functioni

  • electron transfer activity Source: UniProtKB

GO - Biological processi

Keywordsi

Biological processElectron transport, Transport

Enzyme and pathway databases

ReactomeiR-HSA-611105 Respiratory electron transport
R-HSA-8876725 Protein methylation

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

EuPathDBiHostDB:ENSG00000105379.9
HGNCiHGNC:3482 ETFB
MIMi130410 gene
neXtProtiNX_P38117

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

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; decreased protein stability. 1 PublicationCorresponds to variant dbSNP:rs104894678EnsemblClinVar.1
Natural variantiVAR_002369164R → Q in GA2B; reduced electron transfer activity. 1 PublicationCorresponds to variant dbSNP:rs104894677EnsemblClinVar.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
Mutagenesisi200 – 203KAKK → RAKR: Does not abolish electron transfer activity. Abolishes sensitivity to inhibition by lysine methyltransferase ETFBKMT. 1 Publication4
Mutagenesisi200 – 202KAK → RAR: Does not abolish methylation by ETFBKMT. 1 Publication3
Mutagenesisi200K → R: Does not abolish electron transfer activity. Decreases sensitivity to inhibition by lysine methyltransferase ETFBKMT. 1 Publication1
Mutagenesisi203K → R: Does not abolish electron transfer activity. Decreases sensitivity to inhibition by lysine methyltransferase ETFBKMT. 1 Publication1

Keywords - Diseasei

Disease mutation, Glutaricaciduria

Organism-specific databases

DisGeNETi2109
MalaCardsiETFB
MIMi231680 phenotype
OpenTargetsiENSG00000105379
Orphaneti394532 Multiple acyl-CoA dehydrogenase deficiency, mild type
394529 Multiple acyl-CoA dehydrogenase 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.2 Publications

Keywords - PTMi

Acetylation, Methylation, Phosphoprotein

Proteomic databases

EPDiP38117
MaxQBiP38117
PaxDbiP38117
PeptideAtlasiP38117
PRIDEiP38117
ProteomicsDBi55282
55283 [P38117-2]
TopDownProteomicsiP38117-1 [P38117-1]
P38117-2 [P38117-2]

2D gel databases

REPRODUCTION-2DPAGEiIPI00004902
UCD-2DPAGEiP38117

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 Expressed in 236 organ(s), highest expression level in heart left ventricle
CleanExiHS_ETFB
ExpressionAtlasiP38117 baseline and differential
GenevisibleiP38117 HS

Organism-specific databases

HPAiHPA018910
HPA018921
HPA018923

Interactioni

Subunit structurei

Heterodimer composed of ETFA and ETFB (PubMed:25023281, PubMed:25416781, PubMed:8962055, PubMed:15159392, PubMed:15975918). Identified in a complex that contains ETFA, ETFB and ETFRF1 (PubMed:27499296). Interacts with ACADM (PubMed:15159392, PubMed:15975918).6 Publications

Binary interactionsi

Protein-protein interaction databases

BioGridi108410, 37 interactors
ComplexPortaliCPX-2731 Electron transfer flavoprotein complex
DIPiDIP-6162N
IntActiP38117, 31 interactors
MINTiP38117
STRINGi9606.ENSP00000346173

Structurei

Secondary structure

1255
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

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
OMAiRIPNMRG
OrthoDBiEOG091G0KHH
PhylomeDBiP38117
TreeFamiTF314039

Family and domain databases

CDDicd01714 ETF_beta, 1 hit
Gene3Di3.40.50.620, 1 hit
InterProiView protein in InterPro
IPR000049 ET-Flavoprotein_bsu_CS
IPR014730 ETF_a/b_N
IPR012255 ETF_b
IPR033948 ETF_beta_N
IPR014729 Rossmann-like_a/b/a_fold
PANTHERiPTHR21294 PTHR21294, 1 hit
PfamiView protein in Pfam
PF01012 ETF, 1 hit
PIRSFiPIRSF000090 Beta-ETF, 1 hit
SMARTiView protein in SMART
SM00893 ETF, 1 hit
PROSITEiView protein in PROSITE
PS01065 ETF_BETA, 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

This entry has 2 described isoforms and 1 potential isoform that is computationally mapped.Show allAlign All

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

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
M0QY67M0QY67_HUMAN
Electron transfer flavoprotein subu...
ETFB
178Annotation score:

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; decreased protein stability. 1 PublicationCorresponds to variant dbSNP:rs104894678EnsemblClinVar.1
Natural variantiVAR_008548154T → M3 PublicationsCorresponds to variant dbSNP:rs1130426EnsemblClinVar.1
Natural variantiVAR_002369164R → Q in GA2B; reduced electron transfer activity. 1 PublicationCorresponds to variant dbSNP:rs104894677EnsemblClinVar.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

Similar proteinsi

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, 37 interactors
ComplexPortaliCPX-2731 Electron transfer flavoprotein complex
DIPiDIP-6162N
IntActiP38117, 31 interactors
MINTiP38117
STRINGi9606.ENSP00000346173

PTM databases

iPTMnetiP38117
PhosphoSitePlusiP38117
SwissPalmiP38117

Polymorphism and mutation databases

BioMutaiETFB

2D gel databases

REPRODUCTION-2DPAGEiIPI00004902
UCD-2DPAGEiP38117

Proteomic databases

EPDiP38117
MaxQBiP38117
PaxDbiP38117
PeptideAtlasiP38117
PRIDEiP38117
ProteomicsDBi55282
55283 [P38117-2]
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
EuPathDBiHostDB:ENSG00000105379.9
GeneCardsiETFB
HGNCiHGNC:3482 ETFB
HPAiHPA018910
HPA018921
HPA018923
MalaCardsiETFB
MIMi130410 gene
231680 phenotype
neXtProtiNX_P38117
OpenTargetsiENSG00000105379
Orphaneti394532 Multiple acyl-CoA dehydrogenase deficiency, mild type
394529 Multiple acyl-CoA dehydrogenase deficiency, severe neonatal type
PharmGKBiPA27898
GenAtlasiSearch...

Phylogenomic databases

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

Enzyme and pathway databases

ReactomeiR-HSA-611105 Respiratory electron transport
R-HSA-8876725 Protein methylation

Miscellaneous databases

EvolutionaryTraceiP38117
GeneWikiiETFB
GenomeRNAii2109
PROiPR:P38117
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000105379 Expressed in 236 organ(s), highest expression level in heart left ventricle
CleanExiHS_ETFB
ExpressionAtlasiP38117 baseline and differential
GenevisibleiP38117 HS

Family and domain databases

CDDicd01714 ETF_beta, 1 hit
Gene3Di3.40.50.620, 1 hit
InterProiView protein in InterPro
IPR000049 ET-Flavoprotein_bsu_CS
IPR014730 ETF_a/b_N
IPR012255 ETF_b
IPR033948 ETF_beta_N
IPR014729 Rossmann-like_a/b/a_fold
PANTHERiPTHR21294 PTHR21294, 1 hit
PfamiView protein in Pfam
PF01012 ETF, 1 hit
PIRSFiPIRSF000090 Beta-ETF, 1 hit
SMARTiView protein in SMART
SM00893 ETF, 1 hit
PROSITEiView protein in PROSITE
PS01065 ETF_BETA, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiETFB_HUMAN
AccessioniPrimary (citable) accession number: P38117
Secondary accession number(s): A8K766
, B3KNY2, Q6IBH7, Q71RF6, Q9Y3S7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 1, 1994
Last sequence update: January 23, 2007
Last modified: November 7, 2018
This is version 193 of the entry and version 3 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. SIMILARITY comments
    Index of protein domains and families
  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. Human chromosome 19
    Human chromosome 19: entries, gene names and cross-references to MIM
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
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Main funding by: National Institutes of Health

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