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O75380 (NDUS6_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified April 16, 2014. Version 113. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (3) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial
Alternative name(s):
Complex I-13kD-A
Short name=CI-13kD-A
NADH-ubiquinone oxidoreductase 13 kDa-A subunit
Gene names
Name:NDUFS6
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length124 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

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.

Subunit structure

Mammalian complex I is composed of 45 different subunits. This is a component of the iron-sulfur (IP) fragment of the enzyme. Ref.3

Subcellular location

Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.

Sequence similarities

Belongs to the complex I NDUFS6 subunit family.

Ontologies

Keywords
   Biological processElectron transport
Respiratory chain
Transport
   Cellular componentMembrane
Mitochondrion
Mitochondrion inner membrane
   DomainTransit peptide
   PTMAcetylation
   Technical termComplete proteome
Reference proteome
Gene Ontology (GO)
   Biological_processcardiovascular system development

Inferred from electronic annotation. Source: Ensembl

cellular metabolic process

Traceable author statement. Source: Reactome

fatty acid metabolic process

Inferred from electronic annotation. Source: Ensembl

mitochondrial electron transport, NADH to ubiquinone

Non-traceable author statement Ref.1PubMed 9878551. Source: UniProtKB

mitochondrion morphogenesis

Inferred from electronic annotation. Source: Ensembl

multicellular organism growth

Inferred from electronic annotation. Source: Ensembl

multicellular organismal aging

Inferred from electronic annotation. Source: Ensembl

muscle contraction

Inferred from electronic annotation. Source: Ensembl

reproductive system development

Inferred from electronic annotation. Source: Ensembl

respiratory electron transport chain

Traceable author statement. Source: Reactome

small molecule metabolic process

Traceable author statement. Source: Reactome

   Cellular_componentmitochondrial inner membrane

Traceable author statement. Source: Reactome

mitochondrial respiratory chain complex I

Inferred from direct assay Ref.3. Source: UniProtKB

   Molecular_functionNADH dehydrogenase (ubiquinone) activity

Non-traceable author statement Ref.1PubMed 9878551. Source: UniProtKB

electron carrier activity

Non-traceable author statement Ref.1. Source: UniProtKB

Complete GO annotation...

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Transit peptide1 – 2828Mitochondrion By similarity
Chain29 – 12496NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial
PRO_0000020020

Amino acid modifications

Modified residue981N6-acetyllysine By similarity

Sequences

Sequence LengthMass (Da)Tools
O75380 [UniParc].

Last modified November 1, 1998. Version 1.
Checksum: 0A1465160BCA772D

FASTA12413,712
        10         20         30         40         50         60 
MAAAMTFCRL LNRCGEAARS LPLGARCFGV RVSPTGEKVT HTGQVYDDKD YRRIRFVGRQ 

        70         80         90        100        110        120 
KEVNENFAID LIAEQPVSEV ETRVIACDGG GGALGHPKVY INLDKETKTG TCGYCGLQFR 


QHHH 

« 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] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA].
[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 and Skin.
[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]"Initial characterization of the human central proteome."
Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
AF044959 mRNA. Translation: AAC27799.1.
BC038664 mRNA. Translation: AAH38664.1.
BC046155 mRNA. Translation: AAH46155.1.
PIRJE0194.
RefSeqNP_004544.1. NM_004553.4.
UniGeneHs.408257.

3D structure databases

ProteinModelPortalO75380.
SMRO75380. Positions 59-119.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid110805. 26 interactions.
IntActO75380. 6 interactions.
MINTMINT-1427509.
STRING9606.ENSP00000274137.

Chemistry

ChEMBLCHEMBL2363065.
DrugBankDB00157. NADH.

PTM databases

PhosphoSiteO75380.

2D gel databases

UCD-2DPAGEO75380.

Proteomic databases

PaxDbO75380.
PeptideAtlasO75380.
PRIDEO75380.

Protocols and materials databases

DNASU4726.
StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000274137; ENSP00000274137; ENSG00000145494.
GeneID4726.
KEGGhsa:4726.
UCSCuc003jcy.3. human.

Organism-specific databases

CTD4726.
GeneCardsGC05P001801.
HGNCHGNC:7713. NDUFS6.
MIM603848. gene.
neXtProtNX_O75380.
Orphanet2609. Isolated NADH-CoQ reductase deficiency.
PharmGKBPA31523.
GenAtlasSearch...

Phylogenomic databases

eggNOGNOG81252.
HOGENOMHOG000161276.
HOVERGENHBG006549.
InParanoidO75380.
KOK03939.
OMATHTGQAW.
OrthoDBEOG7W41F0.
PhylomeDBO75380.
TreeFamTF315128.

Enzyme and pathway databases

ReactomeREACT_111217. Metabolism.

Gene expression databases

ArrayExpressO75380.
BgeeO75380.
CleanExHS_NDUFS6.
GenevestigatorO75380.

Family and domain databases

Gene3D2.60.260.40. 1 hit.
InterProIPR016668. NADH_Ub_cplx-1_Fe-S_6_mit.
IPR019401. Znf_CHCC.
[Graphical view]
PfamPF10276. zf-CHCC. 1 hit.
[Graphical view]
PIRSFPIRSF016564. CI-13KD-A. 1 hit.
ProtoNetSearch...

Other

ChiTaRSNDUFS6. human.
GeneWikiNDUFS6.
GenomeRNAi4726.
NextBio18226.
PROO75380.
SOURCESearch...

Entry information

Entry nameNDUS6_HUMAN
AccessionPrimary (citable) accession number: O75380
Entry history
Integrated into UniProtKB/Swiss-Prot: December 15, 1998
Last sequence update: November 1, 1998
Last modified: April 16, 2014
This is version 113 of the entry and version 1 of the sequence. [Complete history]
Entry statusReviewed (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.

Relevant documents

SIMILARITY comments

Index of protein domains and families

MIM cross-references

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

Human chromosome 5

Human chromosome 5: entries, gene names and cross-references to MIM