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O75380

- NDUS6_HUMAN

UniProt

O75380 - NDUS6_HUMAN

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
  1. 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

    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

    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

    ArrayExpressiO75380.
    BgeeiO75380.
    CleanExiHS_NDUFS6.
    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. 27 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

    MAAAMTFCRL LNRCGEAARS LPLGARCFGV RVSPTGEKVT HTGQVYDDKD    50
    YRRIRFVGRQ KEVNENFAID LIAEQPVSEV ETRVIACDGG GGALGHPKVY 100
    INLDKETKTG TCGYCGLQFR QHHH 124
    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. 27 interactions.
    IntActi O75380. 6 interactions.
    MINTi MINT-1427509.
    STRINGi 9606.ENSP00000274137.

    Chemistry

    ChEMBLi CHEMBL2363065.
    DrugBanki DB00157. NADH.

    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

    ArrayExpressi O75380.
    Bgeei O75380.
    CleanExi HS_NDUFS6.
    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

    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 1, 2014
    This is version 117 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