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O75380

- NDUS6_HUMAN

UniProt

O75380 - NDUS6_HUMAN

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Protein

NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial

Gene

NDUFS6

Organism
Homo sapiens (Human)
Status
Reviewed - 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

  1. electron carrier activity Source: UniProtKB
  2. NADH dehydrogenase (ubiquinone) activity Source: UniProtKB

GO - Biological processi

  1. cardiovascular system development Source: Ensembl
  2. cellular metabolic process Source: Reactome
  3. fatty acid metabolic process Source: Ensembl
  4. mitochondrial electron transport, NADH to ubiquinone Source: UniProtKB
  5. mitochondrion morphogenesis Source: Ensembl
  6. multicellular organismal aging Source: Ensembl
  7. multicellular organism growth Source: Ensembl
  8. muscle contraction Source: Ensembl
  9. reproductive system development Source: Ensembl
  10. respiratory electron transport chain Source: Reactome
  11. small molecule metabolic process Source: Reactome
Complete GO annotation...

Keywords - Biological processi

Electron transport, Respiratory chain, Transport

Enzyme and pathway databases

ReactomeiREACT_22393. Respiratory electron transport.

Names & Taxonomyi

Protein namesi
Recommended 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 namesi
Name:NDUFS6
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640: Chromosome 5

Organism-specific databases

HGNCiHGNC:7713. NDUFS6.

Subcellular locationi

GO - Cellular componenti

  1. mitochondrial inner membrane Source: Reactome
  2. mitochondrial respiratory chain complex I Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

Orphaneti2609. Isolated NADH-CoQ reductase deficiency.
PharmGKBiPA31523.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 2828MitochondrionBy similarityAdd
BLAST
Chaini29 – 12496NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrialPRO_0000020020Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei98 – 981N6-acetyllysineBy similarity

Keywords - PTMi

Acetylation

Proteomic databases

MaxQBiO75380.
PaxDbiO75380.
PeptideAtlasiO75380.
PRIDEiO75380.

2D gel databases

UCD-2DPAGEO75380.

PTM databases

PhosphoSiteiO75380.

Expressioni

Gene expression databases

BgeeiO75380.
CleanExiHS_NDUFS6.
ExpressionAtlasiO75380. baseline and differential.
GenevestigatoriO75380.

Interactioni

Subunit structurei

Mammalian complex I is composed of 45 different subunits. This is a component of the iron-sulfur (IP) fragment of the enzyme.1 Publication

Protein-protein interaction databases

BioGridi110805. 35 interactions.
IntActiO75380. 6 interactions.
MINTiMINT-1427509.
STRINGi9606.ENSP00000274137.

Structurei

3D structure databases

ProteinModelPortaliO75380.
SMRiO75380. Positions 59-119.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the complex I NDUFS6 subunit family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiNOG81252.
HOGENOMiHOG000161276.
HOVERGENiHBG006549.
InParanoidiO75380.
KOiK03939.
OMAiTHTGQAW.
OrthoDBiEOG7W41F0.
PhylomeDBiO75380.
TreeFamiTF315128.

Family and domain databases

Gene3Di2.60.260.40. 1 hit.
InterProiIPR016668. NADH_Ub_cplx-1_Fe-S_6_mit.
IPR019401. Znf_CHCC.
[Graphical view]
PfamiPF10276. zf-CHCC. 1 hit.
[Graphical view]
PIRSFiPIRSF016564. CI-13KD-A. 1 hit.

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

O75380-1 [UniParc]FASTAAdd to Basket

« Hide

        10         20         30         40         50
MAAAMTFCRL LNRCGEAARS LPLGARCFGV RVSPTGEKVT HTGQVYDDKD
60 70 80 90 100
YRRIRFVGRQ KEVNENFAID LIAEQPVSEV ETRVIACDGG GGALGHPKVY
110 120
INLDKETKTG TCGYCGLQFR QHHH
Length:124
Mass (Da):13,712
Last modified:November 1, 1998 - v1
Checksum:i0A1465160BCA772D
GO

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
AF044959 mRNA. Translation: AAC27799.1.
BC038664 mRNA. Translation: AAH38664.1.
BC046155 mRNA. Translation: AAH46155.1.
CCDSiCCDS3866.1.
PIRiJE0194.
RefSeqiNP_004544.1. NM_004553.4.
UniGeneiHs.408257.

Genome annotation databases

EnsembliENST00000274137; ENSP00000274137; ENSG00000145494.
GeneIDi4726.
KEGGihsa:4726.
UCSCiuc003jcy.3. human.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
AF044959 mRNA. Translation: AAC27799.1 .
BC038664 mRNA. Translation: AAH38664.1 .
BC046155 mRNA. Translation: AAH46155.1 .
CCDSi CCDS3866.1.
PIRi JE0194.
RefSeqi NP_004544.1. NM_004553.4.
UniGenei Hs.408257.

3D structure databases

ProteinModelPortali O75380.
SMRi O75380. Positions 59-119.
ModBasei Search...
MobiDBi Search...

Protein-protein interaction databases

BioGridi 110805. 35 interactions.
IntActi O75380. 6 interactions.
MINTi MINT-1427509.
STRINGi 9606.ENSP00000274137.

Chemistry

ChEMBLi CHEMBL2363065.

PTM databases

PhosphoSitei O75380.

2D gel databases

UCD-2DPAGE O75380.

Proteomic databases

MaxQBi O75380.
PaxDbi O75380.
PeptideAtlasi O75380.
PRIDEi O75380.

Protocols and materials databases

DNASUi 4726.
Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENST00000274137 ; ENSP00000274137 ; ENSG00000145494 .
GeneIDi 4726.
KEGGi hsa:4726.
UCSCi uc003jcy.3. human.

Organism-specific databases

CTDi 4726.
GeneCardsi GC05P001801.
HGNCi HGNC:7713. NDUFS6.
MIMi 603848. gene.
neXtProti NX_O75380.
Orphaneti 2609. Isolated NADH-CoQ reductase deficiency.
PharmGKBi PA31523.
GenAtlasi Search...

Phylogenomic databases

eggNOGi NOG81252.
HOGENOMi HOG000161276.
HOVERGENi HBG006549.
InParanoidi O75380.
KOi K03939.
OMAi THTGQAW.
OrthoDBi EOG7W41F0.
PhylomeDBi O75380.
TreeFami TF315128.

Enzyme and pathway databases

Reactomei REACT_22393. Respiratory electron transport.

Miscellaneous databases

ChiTaRSi NDUFS6. human.
GeneWikii NDUFS6.
GenomeRNAii 4726.
NextBioi 18226.
PROi O75380.
SOURCEi Search...

Gene expression databases

Bgeei O75380.
CleanExi HS_NDUFS6.
ExpressionAtlasi O75380. baseline and differential.
Genevestigatori O75380.

Family and domain databases

Gene3Di 2.60.260.40. 1 hit.
InterProi IPR016668. NADH_Ub_cplx-1_Fe-S_6_mit.
IPR019401. Znf_CHCC.
[Graphical view ]
Pfami PF10276. zf-CHCC. 1 hit.
[Graphical view ]
PIRSFi PIRSF016564. CI-13KD-A. 1 hit.
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  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. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].

Entry informationi

Entry nameiNDUS6_HUMAN
AccessioniPrimary (citable) accession number: O75380
Entry historyi
Integrated into UniProtKB/Swiss-Prot: December 15, 1998
Last sequence update: November 1, 1998
Last modified: October 29, 2014
This is version 118 of the entry and version 1 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 5
    Human chromosome 5: 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

External Data

Dasty 3