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Protein

NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial

Gene

NDUFV3

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

Functioni

Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in 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. May be the terminally assembled subunit of Complex I.1 Publication

GO - Molecular functioni

  • NADH dehydrogenase (ubiquinone) activity Source: ProtInc
  • RNA binding Source: UniProtKB

GO - Biological processi

  • mitochondrial ATP synthesis coupled electron transport Source: CAFA
  • mitochondrial electron transport, NADH to ubiquinone Source: ProtInc
  • mitochondrial respiratory chain complex I assembly Source: Reactome

Keywordsi

Biological processElectron transport, Respiratory chain, Transport

Enzyme and pathway databases

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

Names & Taxonomyi

Protein namesi
Recommended name:
NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial
Alternative name(s):
Complex I-9kD
Short name:
CI-9kD
NADH-ubiquinone oxidoreductase 9 kDa subunit
Renal carcinoma antigen NY-REN-4
Gene namesi
Name:NDUFV3
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 21

Organism-specific databases

EuPathDBiHostDB:ENSG00000160194.17
HGNCiHGNC:7719 NDUFV3
MIMi602184 gene
neXtProtiNX_P56181

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

DisGeNETi4731
OpenTargetsiENSG00000160194
PharmGKBiPA31529

Chemistry databases

ChEMBLiCHEMBL2363065
DrugBankiDB00157 NADH

Polymorphism and mutation databases

BioMutaiNDUFV3

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 34MitochondrionBy similarityAdd BLAST34
ChainiPRO_000002002535 – 108NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrialAdd BLAST74

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei105PhosphoserineCombined sources1
Isoform 2 (identifier: P56181-2)
Modified residuei160PhosphoserineCombined sourcesCurated1
Modified residuei162PhosphoserineCombined sourcesCurated1
Modified residuei164PhosphoserineCombined sourcesCurated1

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiP56181
MaxQBiP56181
PaxDbiP56181
PeptideAtlasiP56181
PRIDEiP56181
ProteomicsDBi56899
56900 [P56181-2]
TopDownProteomicsiP56181-1 [P56181-1]

PTM databases

iPTMnetiP56181
PhosphoSitePlusiP56181

Expressioni

Gene expression databases

BgeeiENSG00000160194
CleanExiHS_NDUFV3
GenevisibleiP56181 HS

Organism-specific databases

HPAiCAB034142
HPA019791
HPA020463
HPA030427

Interactioni

Subunit structurei

Complex I is composed of 45 different subunits. This is a component of the flavoprotein-sulfur (FP) fragment of the enzyme.2 Publications

Protein-protein interaction databases

BioGridi110809, 48 interactors
ComplexPortaliCPX-577 Mitochondrial respiratory chain complex I
CORUMiP56181
IntActiP56181, 28 interactors
MINTiP56181
STRINGi9606.ENSP00000346196

Structurei

Secondary structure

1108
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi83 – 90Combined sources8
Helixi91 – 94Combined sources4

3D structure databases

ProteinModelPortaliP56181
SMRiP56181
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the complex I NDUFV3 subunit family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiENOG410IXG2 Eukaryota
ENOG410YVZC LUCA
GeneTreeiENSGT00390000012196
HOGENOMiHOG000013001
HOVERGENiHBG082013
InParanoidiP56181
KOiK03944
OMAiPFEVKGP
OrthoDBiEOG091G0DNG
PhylomeDBiP56181
TreeFamiTF338771

Family and domain databases

InterProiView protein in InterPro
IPR026193 NDUFV3
PANTHERiPTHR17117 PTHR17117, 2 hits
PfamiView protein in Pfam
PF15880 NDUFV3, 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: P56181-1) [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
MAAPCLLRQG RAGALKTMLQ EAQVFRGLAS TVSLSAESGK SEKGQPQNSK
60 70 80 90 100
KQSPPKKPAP VPAEPFDNTT YKNLQHHDYS TYTFLDLNLE LSKFRMPQPS

SGRESPRH
Length:108
Mass (Da):11,941
Last modified:April 16, 2002 - v2
Checksum:iBD7375474AB0B1EC
GO
Isoform 2 (identifier: P56181-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     56-57: KK → KNVVEPKERG...PGDDRGGTQE

Show »
Length:473
Mass (Da):50,984
Checksum:iBCC3F2E545CA738A
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti9Q → K in CAB56704 (PubMed:9344673).Curated1
Isoform 2 (identifier: P56181-2)
Sequence conflicti415D → N in AAH21217 (PubMed:15489334).Combined sourcesCurated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_03855256 – 57KK → KNVVEPKERGKLLATQTAAE LSKNLSSPSSYPPAVNKGRK VASPSPSGSVLFTDEGVPKF LSRKTLVEFPQKVLSPFRKQ GSDSEARQVGRKVTSPSSSS SSSSSDSESDDEADVSEVTP RVVSKGRGGLRKPEASHSFE NRAPRVTVSAKEKTLLQKPH VDITDPEKPHQPKKKGSPAK PSEGRENARPKTTMPRSQVD EEFLKQSLKEKQLQKTFRLN EIDKESQKPFEVKGPLPVHT KSGLSAPPKGSPAPAVLAEE ARAEGQLQASPPGAAEGHLE KPVPEPQRKAAPPLPRKETS GTQGIEGHLKGGQAIVEDQI PPSNLETVPVENNHGFHEKT AALKLEAEGEAMEDAAAPGD DRGGTQE in isoform 2. 1 Publication2

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X99726, X99727, X99728 Genomic DNA Translation: CAB56704.1
AB038163 Genomic DNA Translation: BAB13732.1
CR542174 mRNA Translation: CAG46971.1
CR542190 mRNA Translation: CAG46987.1
AK289586 mRNA Translation: BAF82275.1
AP001629 Genomic DNA No translation available.
CH471079 Genomic DNA Translation: EAX09524.1
CH471079 Genomic DNA Translation: EAX09526.1
BC021217 mRNA Translation: AAH21217.2
BC033766 mRNA Translation: AAH33766.1
BC054016 mRNA Translation: AAH54016.1
CCDSiCCDS33572.1 [P56181-2]
CCDS33573.1 [P56181-1]
RefSeqiNP_001001503.1, NM_001001503.1 [P56181-1]
NP_066553.3, NM_021075.3 [P56181-2]
UniGeneiHs.473937

Genome annotation databases

EnsembliENST00000340344; ENSP00000342895; ENSG00000160194 [P56181-1]
ENST00000354250; ENSP00000346196; ENSG00000160194 [P56181-2]
GeneIDi4731
KEGGihsa:4731
UCSCiuc002zcm.4 human [P56181-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiNDUV3_HUMAN
AccessioniPrimary (citable) accession number: P56181
Secondary accession number(s): A8K0M1
, J3KNX7, Q6FGD3, Q8WU60, Q9HCR5
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: April 16, 2002
Last modified: July 18, 2018
This is version 146 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. Human chromosome 21
    Human chromosome 21: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  4. SIMILARITY comments
    Index of protein domains and families

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