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Protein

NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial

Gene

NDUFV2

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

Functioni

Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity).By similarity

Catalytic activityi

NADH + ubiquinone + 5 H+(In) = NAD+ + ubiquinol + 4 H+(Out).
NADH + acceptor = NAD+ + reduced acceptor.

Cofactori

[2Fe-2S] clusterCuratedNote: Binds 1 [2Fe-2S] cluster.Curated

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi135Iron-sulfur (2Fe-2S)Sequence analysis1
Metal bindingi140Iron-sulfur (2Fe-2S)Sequence analysis1
Metal bindingi176Iron-sulfur (2Fe-2S)Sequence analysis1
Metal bindingi180Iron-sulfur (2Fe-2S)Sequence analysis1

GO - Molecular functioni

  • 2 iron, 2 sulfur cluster binding Source: UniProtKB-KW
  • electron transfer activity Source: UniProtKB
  • metal ion binding Source: UniProtKB-KW
  • NADH dehydrogenase (ubiquinone) activity Source: UniProtKB

GO - Biological processi

Keywordsi

Molecular functionOxidoreductase, Translocase
Biological processElectron transport, Respiratory chain, Transport
Ligand2Fe-2S, Iron, Iron-sulfur, Metal-binding, NAD, Ubiquinone

Enzyme and pathway databases

ReactomeiR-HSA-611105 Respiratory electron transport
R-HSA-6799198 Complex I biogenesis
SIGNORiP19404

Names & Taxonomyi

Protein namesi
Recommended name:
NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial (EC:1.6.99.3, EC:7.1.1.2)
Alternative name(s):
NADH-ubiquinone oxidoreductase 24 kDa subunit
Gene namesi
Name:NDUFV2
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 18

Organism-specific databases

EuPathDBiHostDB:ENSG00000178127.12
HGNCiHGNC:7717 NDUFV2
MIMi600532 gene
neXtProtiNX_P19404

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

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi4729
MalaCardsiNDUFV2
OpenTargetsiENSG00000178127
Orphaneti2609 Isolated complex I deficiency
255241 Leigh syndrome with leukodystrophy
PharmGKBiPA31527

Chemistry databases

ChEMBLiCHEMBL2363065
DrugBankiDB00157 NADH

Polymorphism and mutation databases

BioMutaiNDUFV2
DMDMi20455499

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 32MitochondrionCombined sourcesAdd BLAST32
ChainiPRO_000002000333 – 249NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrialAdd BLAST217

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei61N6-acetyllysineBy similarity1
Modified residuei193Phosphotyrosine; by SRC1 Publication1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiP19404
MaxQBiP19404
PaxDbiP19404
PeptideAtlasiP19404
PRIDEiP19404
ProteomicsDBi53655
TopDownProteomicsiP19404

2D gel databases

SWISS-2DPAGEiP19404
UCD-2DPAGEiP19404

PTM databases

iPTMnetiP19404
PhosphoSitePlusiP19404

Expressioni

Gene expression databases

BgeeiENSG00000178127 Expressed in 91 organ(s), highest expression level in muscle of leg
CleanExiHS_NDUFV2
ExpressionAtlasiP19404 baseline and differential
GenevisibleiP19404 HS

Organism-specific databases

HPAiHPA003404
HPA077896

Interactioni

Subunit structurei

Complex I is composed of 45 different subunits. This is a component of the flavoprotein-sulfur (FP) fragment of the enzyme.1 Publication

Binary interactionsi

WithEntry#Exp.IntActNotes
HSCBQ8IWL36EBI-713665,EBI-1805738

Protein-protein interaction databases

BioGridi110807, 61 interactors
ComplexPortaliCPX-577 Mitochondrial respiratory chain complex I
CORUMiP19404
IntActiP19404, 46 interactors
MINTiP19404
STRINGi9606.ENSP00000327268

Structurei

Secondary structure

1249
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliP19404
SMRiP19404
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the complex I 24 kDa subunit family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG3196 Eukaryota
COG1905 LUCA
GeneTreeiENSGT00390000017580
HOGENOMiHOG000257748
HOVERGENiHBG029601
InParanoidiP19404
KOiK03943
PhylomeDBiP19404
TreeFamiTF300004

Family and domain databases

CDDicd03064 TRX_Fd_NuoE, 1 hit
InterProiView protein in InterPro
IPR002023 NuoE-like
IPR036249 Thioredoxin-like_sf
PANTHERiPTHR10371 PTHR10371, 1 hit
PIRSFiPIRSF000216 NADH_DH_24kDa, 1 hit
SUPFAMiSSF52833 SSF52833, 1 hit
TIGRFAMsiTIGR01958 nuoE_fam, 1 hit
PROSITEiView protein in PROSITE
PS01099 COMPLEX1_24K, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry has 1 described isoform and 3 potential isoforms that are computationally mapped.Show allAlign All

P19404-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MFFSAALRAR AAGLTAHWGR HVRNLHKTVM QNGAGGALFV HRDTPENNPD
60 70 80 90 100
TPFDFTPENY KRIEAIVKNY PEGHKAAAVL PVLDLAQRQN GWLPISAMNK
110 120 130 140 150
VAEVLQVPPM RVYEVATFYT MYNRKPVGKY HIQVCTTTPC MLRNSDSILE
160 170 180 190 200
AIQKKLGIKV GETTPDKLFT LIEVECLGAC VNAPMVQIND NYYEDLTAKD
210 220 230 240
IEEIIDELKA GKIPKPGPRS GRFSCEPAGG LTSLTEPPKG PGFGVQAGL
Length:249
Mass (Da):27,392
Last modified:May 2, 2002 - v2
Checksum:iAAF46ABB0908B177
GO

Computationally mapped potential isoform sequencesi

There are 3 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
E7EPT4E7EPT4_HUMAN
NADH dehydrogenase [ubiquinone] fla...
NDUFV2
252Annotation score:
J3KRB4J3KRB4_HUMAN
NADH dehydrogenase [ubiquinone] fla...
NDUFV2
39Annotation score:
J3QS34J3QS34_HUMAN
NADH dehydrogenase [ubiquinone] fla...
NDUFV2
21Annotation score:

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_01616729V → A1 PublicationCorresponds to variant dbSNP:rs906807EnsemblClinVar.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M22538 mRNA Translation: AAA75390.1
BC001632 mRNA Translation: AAH01632.1
BC017487 mRNA Translation: AAH17487.1
CCDSiCCDS11842.1
PIRiA30113
RefSeqiNP_066552.2, NM_021074.4
UniGeneiHs.464572

Genome annotation databases

EnsembliENST00000318388; ENSP00000327268; ENSG00000178127
GeneIDi4729
KEGGihsa:4729
UCSCiuc002knu.3 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M22538 mRNA Translation: AAA75390.1
BC001632 mRNA Translation: AAH01632.1
BC017487 mRNA Translation: AAH17487.1
CCDSiCCDS11842.1
PIRiA30113
RefSeqiNP_066552.2, NM_021074.4
UniGeneiHs.464572

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
5XTBelectron microscopy3.40O36-247[»]
5XTDelectron microscopy3.70O36-247[»]
5XTHelectron microscopy3.90O36-247[»]
5XTIelectron microscopy17.40BO/O36-247[»]
ProteinModelPortaliP19404
SMRiP19404
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110807, 61 interactors
ComplexPortaliCPX-577 Mitochondrial respiratory chain complex I
CORUMiP19404
IntActiP19404, 46 interactors
MINTiP19404
STRINGi9606.ENSP00000327268

Chemistry databases

ChEMBLiCHEMBL2363065
DrugBankiDB00157 NADH

PTM databases

iPTMnetiP19404
PhosphoSitePlusiP19404

Polymorphism and mutation databases

BioMutaiNDUFV2
DMDMi20455499

2D gel databases

SWISS-2DPAGEiP19404
UCD-2DPAGEiP19404

Proteomic databases

EPDiP19404
MaxQBiP19404
PaxDbiP19404
PeptideAtlasiP19404
PRIDEiP19404
ProteomicsDBi53655
TopDownProteomicsiP19404

Protocols and materials databases

DNASUi4729
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000318388; ENSP00000327268; ENSG00000178127
GeneIDi4729
KEGGihsa:4729
UCSCiuc002knu.3 human

Organism-specific databases

CTDi4729
DisGeNETi4729
EuPathDBiHostDB:ENSG00000178127.12
GeneCardsiNDUFV2
HGNCiHGNC:7717 NDUFV2
HPAiHPA003404
HPA077896
MalaCardsiNDUFV2
MIMi600532 gene
neXtProtiNX_P19404
OpenTargetsiENSG00000178127
Orphaneti2609 Isolated complex I deficiency
255241 Leigh syndrome with leukodystrophy
PharmGKBiPA31527
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3196 Eukaryota
COG1905 LUCA
GeneTreeiENSGT00390000017580
HOGENOMiHOG000257748
HOVERGENiHBG029601
InParanoidiP19404
KOiK03943
PhylomeDBiP19404
TreeFamiTF300004

Enzyme and pathway databases

ReactomeiR-HSA-611105 Respiratory electron transport
R-HSA-6799198 Complex I biogenesis
SIGNORiP19404

Miscellaneous databases

ChiTaRSiNDUFV2 human
GeneWikiiNDUFV2
GenomeRNAii4729
PROiPR:P19404
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000178127 Expressed in 91 organ(s), highest expression level in muscle of leg
CleanExiHS_NDUFV2
ExpressionAtlasiP19404 baseline and differential
GenevisibleiP19404 HS

Family and domain databases

CDDicd03064 TRX_Fd_NuoE, 1 hit
InterProiView protein in InterPro
IPR002023 NuoE-like
IPR036249 Thioredoxin-like_sf
PANTHERiPTHR10371 PTHR10371, 1 hit
PIRSFiPIRSF000216 NADH_DH_24kDa, 1 hit
SUPFAMiSSF52833 SSF52833, 1 hit
TIGRFAMsiTIGR01958 nuoE_fam, 1 hit
PROSITEiView protein in PROSITE
PS01099 COMPLEX1_24K, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiNDUV2_HUMAN
AccessioniPrimary (citable) accession number: P19404
Secondary accession number(s): Q9BV41
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1990
Last sequence update: May 2, 2002
Last modified: November 7, 2018
This is version 203 of the entry and version 2 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 18
    Human chromosome 18: entries, gene names and cross-references to MIM
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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