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Reviewed, UniProtKB/Swiss-Prot O75489 (NDUS3_HUMAN)

Last modified November 25, 2008. Version 78. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (5) | Third-party data | Customize display text xml rdf/xml gff fasta
Names and origin · Protein attributes · General annotation (Comments) · Ontologies · Binary interactions · Sequence annotation (Features) · Sequences · References · Cross-references · Entry information · Relevant documents

Names and origin

Protein namesRecommended name:
    NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial
    EC=1.6.5.3
    EC=1.6.99.3
Alternative name(s):
    NADH-ubiquinone oxidoreductase 30 kDa subunit
    Complex I-30kD
      Short name=CI-30kD
Gene names
Name: NDUFS3
OrganismHomo sapiens (Human)
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length264 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at protein level.

General annotation (Comments)

Function

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.

Catalytic activity

NADH + ubiquinone = NAD(+) + ubiquinol.

NADH + acceptor = NAD(+) + reduced acceptor.

Subunit structure

Mammalian complex I is composed of 45 different subunits.

Subcellular location

Mitochondrion inner membrane.

Sequence similarities

Belongs to the complex I 30 kDa subunit family.

Binary interactions

With

Entry

#Exp.

IntAct

Notes

GZMAP125441EBI-1224896,EBI-519800

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Transit peptide1 – 3636Mitochondrion By similarity
Chain37 – 264228NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial
PRO_0000019998

Natural variations

Natural variant2491P → Q: dbSNP rs9600.
VAR_012036

Experimental info

Sequence conflict1 – 77MAAAAVA → MAAGRY Ref.3

Sequences

Sequence LengthMass (Da)Tools
O75489-1 [UniParc].

Last modified November 1, 1998. Version 1.
Checksum: C058D62779BEF17B

FASTA26430,242
        10         20         30         40         50         60 
MAAAAVARLW WRGILGASAL TRGTGRPSVL LLPVRRESAG ADTRPTVRPR NDVAHKQLSA 

        70         80         90        100        110        120 
FGEYVAEILP KYVQQVQVSC FNELEVCIHP DGVIPVLTFL RDHTNAQFKS LVDLTAVDVP 

       130        140        150        160        170        180 
TRQNRFEIVY NLLSLRFNSR IRVKTYTDEL TPIESAVSVF KAANWYEREI WDMFGVFFAN 

       190        200        210        220        230        240 
HPDLRRILTD YGFEGHPFRK DFPLSGYVEL RYDDEVKRVV AEPVELAQEF RKFDLNSPWE 

       250        260 
AFPVYRQPPE SLKLEAGDKK PDAK 

« Hide

References

« Hide 'large scale' references
[1]"cDNA sequence and chromosomal localization of the remaining three human nuclear encoded iron sulphur protein (IP) subunits of complex I: the human IP fraction is completed."
Loeffen J., van den Heuvel L., Smeets R., Triepels R., Sengers R., Trijbels F., Smeitink J.
Biochem. Biophys. Res. Commun. 247:751-758(1998) [PubMed: 9647766] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA].
[2]"Human NDUFS3 gene coding for the 30-kDa subunit of mitochondrial Complex I: genomic organization and expression."
Procaccio V., Lescuyer P., Bourges I., Beugnot R., Duborjal H., Depetris D., Mousson B., Montfort M.F., Smeets H., De Coo R., Issartel J.P.
Mamm. Genome 11:808-810(2000) [PubMed: 10967146] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
[3]"Gene expression profiling in the human hypothalamus-pituitary-adrenal axis and full-length cDNA cloning."
Hu R.-M., Han Z.-G., Song H.-D., Peng Y.-D., Huang Q.-H., Ren S.-X., Gu Y.-J., Huang C.-H., Li Y.-B., Jiang C.-L., Fu G., Zhang Q.-H., Gu B.-W., Dai M., Mao Y.-F., Gao G.-F., Rong R., Ye M. expand/collapse author list , Zhou J., Xu S.-H., Gu J., Shi J.-X., Jin W.-R., Zhang C.-K., Wu T.-M., Huang G.-Y., Chen Z., Chen M.-D., Chen J.-L.
Proc. Natl. Acad. Sci. U.S.A. 97:9543-9548(2000) [PubMed: 10931946] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
Tissue: Pituitary.
[4]"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: 15489334] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
Tissue: Skin.
[5]Lubec G., Vishwanath V.
Submitted (MAR-2007) to UniProtKB
Cited for: PROTEIN SEQUENCE OF 126-136 AND 187-199, MASS SPECTROMETRY.
Tissue: Brain and Cajal-Retzius cell.

Cross-references

Sequence databases

AF067139 mRNA. Translation: AAC27451.1.
AF200954 Genomic DNA. Translation: AAG17541.1.
AF100743 mRNA. Translation: AAD40386.1.
BC000617 mRNA. Translation: AAH00617.1.
PIRJE0195.
RefSeqNP_004542.1.
UniGeneHs.502528

3D structure databases

ModBaseSearch...

Protein-protein interaction databases

IntActO75489.

PTM databases

PhosphoSiteO75489.

2-D gel databases

REPRODUCTION-2DPAGEIPI00025796.
O75489.

Proteomic databases

PeptideAtlasO75489.

Genome annotation databases

EnsemblENSG00000213619. Homo sapiens. [Contig view]
GeneID4722.
KEGGhsa:4722.
NMPDRfig|9606.3.peg.5556.

Organism-specific databases

H-InvDBHIX0009620.
HIX0079399.
HGNCHGNC:7710. NDUFS3.
HPAHPA004484.
MIM603846. gene.
Orphanet506. Leigh syndrome.
2609. NADH-CoQ reductase deficiency.
PharmGKBPA31520.
GenAtlasSearch...
GeneCardsSearch...

Phylogenomic databases

HOVERGENO75489.

Enzyme and pathway databases

ReactomeREACT_6305. Electron Transport Chain.

Gene expression databases

ArrayExpressO75489.
CleanExHS_NDUFS3.
GermOnlineENSG00000110536. Homo sapiens.

Family and domain databases

InterProIPR010218. NADH_DH_csu.
IPR001268. NADH_DHase_Ub_30kDa_su.
[Graphical view]
PfamPF00329. Complex1_30kDa. 1 hit.
[Graphical view]
ProDomPD001581. Complex1_30K. 1 hit.
[Graphical view] [Entries sharing at least one domain]
TIGRFAMsTIGR01961. NuoC_fam. 1 hit.
PROSITEPS00542. COMPLEX1_30K. 1 hit.
[Graphical view]
ProtoNetSearch...

Other Resources

DrugBankDB00157. NADH.
NextBio18210.
SOURCESearch...

Entry information

Entry nameNDUS3_HUMAN
AccessionPrimary (citable) accession number: O75489
Secondary accession number(s): Q9UNQ8
Entry history
Integrated into UniProtKB/Swiss-Prot: December 15, 1998
Last sequence update: November 1, 1998
Last modified: November 25, 2008
This is version 78 of the entry and version 1 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation projectHPI (Human Proteome Initiative)

Relevant documents

Human chromosome 11

Human chromosome 11: entries, gene names and cross-references to MIM

Human entries with polymorphisms or disease mutations

List of human entries with polymorphisms or disease mutations

Human polymorphisms and disease mutations

Index of human polymorphisms and disease mutations

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

SIMILARITY comments

Index of protein domains and families

Names and origin · Protein attributes · General annotation (Comments) · Ontologies · Binary interactions · Sequence annotation (Features) · Sequences · References · Cross-references · Entry information · Relevant documents