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Protein

NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial

Gene

NDUFS3

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.

GO - Molecular functioni

  • electron transfer activity Source: UniProtKB
  • NADH dehydrogenase (ubiquinone) activity Source: GO_Central
  • NADH dehydrogenase activity Source: UniProtKB

GO - Biological processi

  • cellular respiration Source: GO_Central
  • mitochondrial electron transport, NADH to ubiquinone Source: UniProtKB
  • mitochondrial respiratory chain complex I assembly Source: Reactome
  • negative regulation of cell growth Source: UniProtKB
  • negative regulation of intrinsic apoptotic signaling pathway Source: UniProtKB
  • reactive oxygen species metabolic process Source: UniProtKB
  • substantia nigra development Source: UniProtKB

Keywordsi

Molecular functionOxidoreductase
Biological processElectron transport, Respiratory chain, Transport
LigandNAD, Ubiquinone

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] iron-sulfur protein 3, mitochondrial (EC:1.6.5.3, EC:1.6.99.3)
Alternative name(s):
Complex I-30kD
Short name:
CI-30kD
NADH-ubiquinone oxidoreductase 30 kDa subunit
Gene namesi
Name:NDUFS3
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 11

Organism-specific databases

EuPathDBiHostDB:ENSG00000213619.9
HGNCiHGNC:7710 NDUFS3
MIMi603846 gene
neXtProtiNX_O75489

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

DisGeNETi4722
MalaCardsiNDUFS3
OpenTargetsiENSG00000213619
Orphaneti2609 Isolated NADH-CoQ reductase deficiency
255241 Leigh syndrome with leukodystrophy
PharmGKBiPA31520

Chemistry databases

ChEMBLiCHEMBL2363065
DrugBankiDB00997 Doxorubicin
DB00157 NADH

Polymorphism and mutation databases

BioMutaiNDUFS3

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 36Mitochondrion1 PublicationAdd BLAST36
ChainiPRO_000001999837 – 264NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrialAdd BLAST228

Proteomic databases

EPDiO75489
MaxQBiO75489
PaxDbiO75489
PeptideAtlasiO75489
PRIDEiO75489
TopDownProteomicsiO75489-1 [O75489-1]

2D gel databases

REPRODUCTION-2DPAGEiIPI00025796
O75489

PTM databases

CarbonylDBiO75489
iPTMnetiO75489
PhosphoSitePlusiO75489
SwissPalmiO75489

Expressioni

Gene expression databases

BgeeiENSG00000213619
CleanExiHS_NDUFS3
ExpressionAtlasiO75489 baseline and differential
GenevisibleiO75489 HS

Organism-specific databases

HPAiHPA004484

Interactioni

Subunit structurei

Mammalian complex I is composed of 45 different subunits. Interacts with NDUFAF3.2 Publications

Binary interactionsi

Show more details

Protein-protein interaction databases

BioGridi110801, 160 interactors
CORUMiO75489
IntActiO75489, 64 interactors
MINTiO75489
STRINGi9606.ENSP00000263774

Structurei

Secondary structure

1264
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi45 – 47Combined sources3
Helixi52 – 68Combined sources17
Turni70 – 72Combined sources3
Beta strandi76 – 78Combined sources3
Beta strandi84 – 87Combined sources4
Helixi90 – 92Combined sources3
Helixi93 – 101Combined sources9
Beta strandi113 – 115Combined sources3
Beta strandi120 – 124Combined sources5
Beta strandi129 – 134Combined sources6
Turni135 – 138Combined sources4
Beta strandi139 – 144Combined sources6
Turni156 – 160Combined sources5
Helixi162 – 164Combined sources3
Helixi165 – 172Combined sources8
Beta strandi178 – 180Combined sources3
Beta strandi187 – 189Combined sources3
Beta strandi207 – 213Combined sources7
Turni214 – 217Combined sources4
Beta strandi218 – 223Combined sources6

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
5XTBelectron microscopy3.40P43-250[»]
5XTDelectron microscopy3.70P43-250[»]
5XTHelectron microscopy3.90P43-250[»]
5XTIelectron microscopy17.40BP/P43-250[»]
ProteinModelPortaliO75489
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the complex I 30 kDa subunit family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG1713 Eukaryota
COG0852 LUCA
GeneTreeiENSGT00390000017480
HOGENOMiHOG000009797
HOVERGENiHBG000450
InParanoidiO75489
KOiK03936
OMAiAECLPKY
OrthoDBiEOG091G0AFP
PhylomeDBiO75489
TreeFamiTF314794

Family and domain databases

Gene3Di3.30.460.80, 1 hit
HAMAPiMF_01357 NDH1_NuoC, 1 hit
InterProiView protein in InterPro
IPR010218 NADH_DH_suC
IPR037232 NADH_quin_OxRdtase_su_C/D-like
IPR001268 NADH_UbQ_OxRdtase_30kDa_su
IPR020396 NADH_UbQ_OxRdtase_CS
PfamiView protein in Pfam
PF00329 Complex1_30kDa, 1 hit
ProDomiView protein in ProDom or Entries sharing at least one domain
PD001581 NADH_UbQ_OxRdtase_30kDa_su, 1 hit
SUPFAMiSSF143243 SSF143243, 1 hit
TIGRFAMsiTIGR01961 NuoC_fam, 1 hit
PROSITEiView protein in PROSITE
PS00542 COMPLEX1_30K, 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: O75489-1) [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
MAAAAVARLW WRGILGASAL TRGTGRPSVL LLPVRRESAG ADTRPTVRPR
60 70 80 90 100
NDVAHKQLSA FGEYVAEILP KYVQQVQVSC FNELEVCIHP DGVIPVLTFL
110 120 130 140 150
RDHTNAQFKS LVDLTAVDVP TRQNRFEIVY NLLSLRFNSR IRVKTYTDEL
160 170 180 190 200
TPIESAVSVF KAANWYEREI WDMFGVFFAN HPDLRRILTD YGFEGHPFRK
210 220 230 240 250
DFPLSGYVEL RYDDEVKRVV AEPVELAQEF RKFDLNSPWE AFPVYRQPPE
260
SLKLEAGDKK PDAK
Length:264
Mass (Da):30,242
Last modified:November 1, 1998 - v1
Checksum:iC058D62779BEF17B
GO
Isoform 2 (identifier: O75489-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     128-132: IVYNL → VSWEI
     133-264: Missing.

Note: No experimental confirmation available.
Show »
Length:132
Mass (Da):14,679
Checksum:iF56D4EA3021A11EF
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti1 – 7MAAAAVA → MAAGRY (PubMed:10931946).Curated7

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_012036249P → Q. Corresponds to variant dbSNP:rs9600Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_057065128 – 132IVYNL → VSWEI in isoform 2. 1 Publication5
Alternative sequenceiVSP_057066133 – 264Missing in isoform 2. 1 PublicationAdd BLAST132

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF067139 mRNA Translation: AAC27451.1
AF200954 Genomic DNA Translation: AAG17541.1
AF100743 mRNA Translation: AAD40386.1
AK294167 mRNA Translation: BAG57489.1
AK313802 mRNA Translation: BAG36538.1
AC090559 Genomic DNA No translation available.
AC104942 Genomic DNA No translation available.
CH471064 Genomic DNA Translation: EAW67895.1
BC000617 mRNA Translation: AAH00617.1
CCDSiCCDS7941.1 [O75489-1]
PIRiJE0195
RefSeqiNP_004542.1, NM_004551.2 [O75489-1]
UniGeneiHs.502528

Genome annotation databases

EnsembliENST00000263774; ENSP00000263774; ENSG00000213619 [O75489-1]
ENST00000534716; ENSP00000434970; ENSG00000213619 [O75489-2]
GeneIDi4722
KEGGihsa:4722
UCSCiuc001nga.3 human [O75489-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Entry informationi

Entry nameiNDUS3_HUMAN
AccessioniPrimary (citable) accession number: O75489
Secondary accession number(s): B2R9J1, B4DFM8, Q9UNQ8
Entry historyiIntegrated into UniProtKB/Swiss-Prot: December 15, 1998
Last sequence update: November 1, 1998
Last modified: May 23, 2018
This is version 180 of the entry and version 1 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, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 11
    Human chromosome 11: 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. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. SIMILARITY comments
    Index of protein domains and families
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