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Protein

NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial

Gene

NDUFV2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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)LengthDescriptionGraphical viewFeature identifierActions
Metal bindingi135 – 1351Iron-sulfur (2Fe-2S)Sequence Analysis
Metal bindingi140 – 1401Iron-sulfur (2Fe-2S)Sequence Analysis
Metal bindingi176 – 1761Iron-sulfur (2Fe-2S)Sequence Analysis
Metal bindingi180 – 1801Iron-sulfur (2Fe-2S)Sequence Analysis

GO - Molecular functioni

  1. 2 iron, 2 sulfur cluster binding Source: UniProtKB-KW
  2. electron carrier activity Source: UniProtKB
  3. metal ion binding Source: UniProtKB-KW
  4. NADH dehydrogenase (ubiquinone) activity Source: UniProtKB

GO - Biological processi

  1. cardiac muscle tissue development Source: UniProtKB
  2. cellular metabolic process Source: Reactome
  3. mitochondrial electron transport, NADH to ubiquinone Source: UniProtKB
  4. nervous system development Source: UniProtKB
  5. respiratory electron transport chain Source: Reactome
  6. small molecule metabolic process Source: Reactome
Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Biological processi

Electron transport, Respiratory chain, Transport

Keywords - Ligandi

2Fe-2S, Iron, Iron-sulfur, Metal-binding, NAD, Ubiquinone

Enzyme and pathway databases

ReactomeiREACT_22393. Respiratory electron transport.

Names & Taxonomyi

Protein namesi
Recommended name:
NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial (EC:1.6.5.3, EC:1.6.99.3)
Alternative name(s):
NADH-ubiquinone oxidoreductase 24 kDa subunit
Gene namesi
Name:NDUFV2
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640 Componenti: Chromosome 18

Organism-specific databases

HGNCiHGNC:7717. NDUFV2.

Subcellular locationi

GO - Cellular componenti

  1. mitochondrial inner membrane Source: Reactome
  2. mitochondrial respiratory chain complex I Source: UniProtKB
  3. mitochondrion Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

Orphaneti2609. Isolated NADH-CoQ reductase deficiency.
PharmGKBiPA31527.

Polymorphism and mutation databases

BioMutaiNDUFV2.
DMDMi20455499.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 3232MitochondrionAdd
BLAST
Chaini33 – 249217NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrialPRO_0000020003Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei61 – 611N6-acetyllysineBy similarity
Modified residuei193 – 1931Phosphotyrosine; by SRC1 Publication

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

MaxQBiP19404.
PaxDbiP19404.
PRIDEiP19404.

2D gel databases

SWISS-2DPAGEP19404.
UCD-2DPAGEP19404.

PTM databases

PhosphoSiteiP19404.

Expressioni

Gene expression databases

BgeeiP19404.
CleanExiHS_NDUFV2.
ExpressionAtlasiP19404. baseline.
GenevestigatoriP19404.

Organism-specific databases

HPAiHPA003404.

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

Protein-protein interaction databases

BioGridi110807. 32 interactions.
IntActiP19404. 8 interactions.
MINTiMINT-4991740.
STRINGi9606.ENSP00000327268.

Structurei

3D structure databases

ProteinModelPortaliP19404.
SMRiP19404. Positions 63-212.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the complex I 24 kDa subunit family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiCOG1905.
GeneTreeiENSGT00390000017580.
HOGENOMiHOG000257748.
HOVERGENiHBG029601.
InParanoidiP19404.
KOiK03943.
PhylomeDBiP19404.
TreeFamiTF300004.

Family and domain databases

Gene3Di3.40.30.10. 1 hit.
InterProiIPR002023. NuoE-like.
IPR012336. Thioredoxin-like_fold.
[Graphical view]
PIRSFiPIRSF000216. NADH_DH_24kDa. 1 hit.
SUPFAMiSSF52833. SSF52833. 1 hit.
TIGRFAMsiTIGR01958. nuoE_fam. 1 hit.
PROSITEiPS01099. COMPLEX1_24K. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

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

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

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti29 – 291V → A.1 Publication
Corresponds to variant rs906807 [ dbSNP | Ensembl ].
VAR_016167

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.

Polymorphism and mutation databases

BioMutaiNDUFV2.

Keywords - Coding sequence diversityi

Polymorphism

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

ProteinModelPortaliP19404.
SMRiP19404. Positions 63-212.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110807. 32 interactions.
IntActiP19404. 8 interactions.
MINTiMINT-4991740.
STRINGi9606.ENSP00000327268.

Chemistry

ChEMBLiCHEMBL2363065.

PTM databases

PhosphoSiteiP19404.

Polymorphism and mutation databases

BioMutaiNDUFV2.
DMDMi20455499.

2D gel databases

SWISS-2DPAGEP19404.
UCD-2DPAGEP19404.

Proteomic databases

MaxQBiP19404.
PaxDbiP19404.
PRIDEiP19404.

Protocols and materials databases

DNASUi4729.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

CTDi4729.
GeneCardsiGC18P009092.
HGNCiHGNC:7717. NDUFV2.
HPAiHPA003404.
MIMi600532. gene.
neXtProtiNX_P19404.
Orphaneti2609. Isolated NADH-CoQ reductase deficiency.
PharmGKBiPA31527.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiCOG1905.
GeneTreeiENSGT00390000017580.
HOGENOMiHOG000257748.
HOVERGENiHBG029601.
InParanoidiP19404.
KOiK03943.
PhylomeDBiP19404.
TreeFamiTF300004.

Enzyme and pathway databases

ReactomeiREACT_22393. Respiratory electron transport.

Miscellaneous databases

ChiTaRSiNDUFV2. human.
GeneWikiiNDUFV2.
GenomeRNAii4729.
NextBioi18234.
PROiP19404.
SOURCEiSearch...

Gene expression databases

BgeeiP19404.
CleanExiHS_NDUFV2.
ExpressionAtlasiP19404. baseline.
GenevestigatoriP19404.

Family and domain databases

Gene3Di3.40.30.10. 1 hit.
InterProiIPR002023. NuoE-like.
IPR012336. Thioredoxin-like_fold.
[Graphical view]
PIRSFiPIRSF000216. NADH_DH_24kDa. 1 hit.
SUPFAMiSSF52833. SSF52833. 1 hit.
TIGRFAMsiTIGR01958. nuoE_fam. 1 hit.
PROSITEiPS01099. COMPLEX1_24K. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "Mitochondrial NADH-ubiquinone reductase: complementary DNA sequences of import precursors of the bovine and human 24-kDa subunit."
    Pilkington S.J., Walker J.E.
    Biochemistry 28:3257-3264(1989) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA], VARIANT ALA-29.
  2. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Tissue: Lung.
  3. "The subunit composition of the human NADH dehydrogenase obtained by rapid one-step immunopurification."
    Murray J., Zhang B., Taylor S.W., Oglesbee D., Fahy E., Marusich M.F., Ghosh S.S., Capaldi R.A.
    J. Biol. Chem. 278:13619-13622(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: IDENTIFICATION IN THE NADH-UBIQUINONE OXIDOREDUCTASE COMPLEX, IDENTIFICATION BY MASS SPECTROMETRY.
  4. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  5. "Mitochondrial c-Src regulates cell survival through phosphorylation of respiratory chain components."
    Ogura M., Yamaki J., Homma M.K., Homma Y.
    Biochem. J. 447:281-289(2012) [PubMed] [Europe PMC] [Abstract]
    Cited for: PHOSPHORYLATION AT TYR-193.
  6. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
    Bian Y., Song C., Cheng K., Dong M., Wang F., Huang J., Sun D., Wang L., Ye M., Zou H.
    J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
    Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.

Entry informationi

Entry nameiNDUV2_HUMAN
AccessioniPrimary (citable) accession number: P19404
Secondary accession number(s): Q9BV41
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 1, 1990
Last sequence update: May 2, 2002
Last modified: April 29, 2015
This is version 168 of the entry and version 2 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

Complete proteome, Reference proteome

Documents

  1. Human chromosome 18
    Human chromosome 18: 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. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

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 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.