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Protein

NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial

Gene

NDUFV3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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.

GO - Molecular functioni

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

GO - Biological processi

Complete GO annotation...

Keywords - Biological processi

Electron transport, Respiratory chain, Transport

Enzyme and pathway databases

BioCyciZFISH:HS08459-MONOMER.
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

HGNCiHGNC:7719. NDUFV3.

Subcellular locationi

GO - Cellular componenti

  • mitochondrial inner membrane Source: Reactome
  • mitochondrial respiratory chain complex I Source: UniProtKB
  • mitochondrion Source: UniProtKB
  • nucleoplasm Source: HPA
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi4731.
OpenTargetsiENSG00000160194.
PharmGKBiPA31529.

Chemistry databases

ChEMBLiCHEMBL2363065.

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.
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.1 Publication

Protein-protein interaction databases

BioGridi110809. 43 interactors.
IntActiP56181. 28 interactors.
MINTiMINT-4535662.
STRINGi9606.ENSP00000346196.

Structurei

3D structure databases

ProteinModelPortaliP56181.
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.
OMAiSQKPFEV.
OrthoDBiEOG091G0DNG.
PhylomeDBiP56181.
TreeFamiTF338771.

Family and domain databases

InterProiIPR026193. NDUFV3.
[Graphical view]
PANTHERiPTHR17117. PTHR17117. 1 hit.
PfamiPF15880. NDUFV3. 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: 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

Cross-referencesi

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.

3D structure databases

ProteinModelPortaliP56181.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110809. 43 interactors.
IntActiP56181. 28 interactors.
MINTiMINT-4535662.
STRINGi9606.ENSP00000346196.

Chemistry databases

ChEMBLiCHEMBL2363065.

PTM databases

iPTMnetiP56181.
PhosphoSitePlusiP56181.

Polymorphism and mutation databases

BioMutaiNDUFV3.

Proteomic databases

EPDiP56181.
MaxQBiP56181.
PaxDbiP56181.
PeptideAtlasiP56181.
PRIDEiP56181.
TopDownProteomicsiP56181-1. [P56181-1]

Protocols and materials databases

DNASUi4731.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

CTDi4731.
DisGeNETi4731.
GeneCardsiNDUFV3.
HGNCiHGNC:7719. NDUFV3.
HPAiCAB034142.
HPA019791.
HPA020463.
HPA030427.
MIMi602184. gene.
neXtProtiNX_P56181.
OpenTargetsiENSG00000160194.
PharmGKBiPA31529.
GenAtlasiSearch...

Phylogenomic databases

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

Enzyme and pathway databases

BioCyciZFISH:HS08459-MONOMER.
ReactomeiR-HSA-611105. Respiratory electron transport.
R-HSA-6799198. Complex I biogenesis.

Miscellaneous databases

ChiTaRSiNDUFV3. human.
GeneWikiiNDUFV3.
GenomeRNAii4731.
PROiP56181.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000160194.
CleanExiHS_NDUFV3.
GenevisibleiP56181. HS.

Family and domain databases

InterProiIPR026193. NDUFV3.
[Graphical view]
PANTHERiPTHR17117. PTHR17117. 1 hit.
PfamiPF15880. NDUFV3. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiNDUV3_HUMAN
AccessioniPrimary (citable) accession number: P56181
Secondary accession number(s): A8K0M1
, J3KNX7, Q6FGD3, Q8WU60, Q9HCR5
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: April 16, 2002
Last modified: November 30, 2016
This is version 134 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 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. 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.