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Q16795

- NDUA9_HUMAN

UniProt

Q16795 - NDUA9_HUMAN

Protein

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial

Gene

NDUFA9

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 126 (01 Oct 2014)
      Sequence version 2 (01 Nov 1997)
      Previous versions | rss
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    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.

    Cofactori

    Binds 1 FAD per subunit.

    GO - Molecular functioni

    1. coenzyme binding Source: InterPro
    2. NADH dehydrogenase (ubiquinone) activity Source: UniProtKB
    3. NADH dehydrogenase activity Source: UniProtKB
    4. protein binding Source: IntAct
    5. protein complex binding Source: MGI

    GO - Biological processi

    1. cellular metabolic process Source: Reactome
    2. mitochondrial electron transport, NADH to ubiquinone Source: UniProtKB
    3. respiratory electron transport chain Source: Reactome
    4. small molecule metabolic process Source: Reactome
    5. sodium ion transport Source: UniProtKB

    Keywords - Biological processi

    Electron transport, Respiratory chain, Transport

    Keywords - Ligandi

    FAD, Flavoprotein

    Enzyme and pathway databases

    ReactomeiREACT_22393. Respiratory electron transport.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial
    Alternative name(s):
    Complex I-39kD
    Short name:
    CI-39kD
    NADH-ubiquinone oxidoreductase 39 kDa subunit
    Gene namesi
    Name:NDUFA9
    Synonyms:NDUFS2L
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    ProteomesiUP000005640: Chromosome 12

    Organism-specific databases

    HGNCiHGNC:7693. NDUFA9.

    Subcellular locationi

    GO - Cellular componenti

    1. mitochondrial inner membrane Source: Reactome
    2. mitochondrial matrix Source: UniProtKB
    3. mitochondrial membrane Source: UniProtKB
    4. mitochondrial respiratory chain complex I Source: UniProtKB
    5. nucleus Source: UniProt

    Keywords - Cellular componenti

    Mitochondrion

    Pathology & Biotechi

    Organism-specific databases

    Orphaneti2609. Isolated NADH-CoQ reductase deficiency.
    PharmGKBiPA31499.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transit peptidei1 – 3535MitochondrionBy similarityAdd
    BLAST
    Chaini36 – 377342NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrialPRO_0000019992Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei175 – 1751N6-succinyllysineBy similarity
    Modified residuei189 – 1891N6-acetyllysineBy similarity
    Modified residuei370 – 3701N6-acetyllysineBy similarity

    Post-translational modificationi

    Acetylated on lysine residues. BLOC1S1 is required for acetylation.1 Publication

    Keywords - PTMi

    Acetylation

    Proteomic databases

    MaxQBiQ16795.
    PaxDbiQ16795.
    PRIDEiQ16795.

    PTM databases

    PhosphoSiteiQ16795.

    Expressioni

    Gene expression databases

    ArrayExpressiQ16795.
    BgeeiQ16795.
    CleanExiHS_NDUFA9.
    GenevestigatoriQ16795.

    Organism-specific databases

    HPAiHPA042268.

    Interactioni

    Subunit structurei

    Complex I is composed of 45 different subunits. This a component of the hydrophobic protein fraction. Interacts with BLOC1S1. Interacts with SLC2A4 By similarity.By similarity

    Binary interactionsi

    WithEntry#Exp.IntActNotes
    BLOC1S1P785373EBI-1045087,EBI-348630
    NOA1Q8NC602EBI-1045087,EBI-717871

    Protein-protein interaction databases

    BioGridi110784. 23 interactions.
    DIPiDIP-38526N.
    IntActiQ16795. 15 interactions.
    MINTiMINT-3033599.
    STRINGi9606.ENSP00000266544.

    Structurei

    3D structure databases

    ProteinModelPortaliQ16795.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Sequence similaritiesi

    Belongs to the complex I NDUFA9 subunit family.Curated

    Keywords - Domaini

    Transit peptide

    Phylogenomic databases

    eggNOGiCOG0702.
    HOGENOMiHOG000168008.
    HOVERGENiHBG006546.
    InParanoidiQ16795.
    KOiK03953.
    OMAiIFLEWNG.
    PhylomeDBiQ16795.
    TreeFamiTF105961.

    Family and domain databases

    Gene3Di3.40.50.720. 1 hit.
    InterProiIPR001509. Epimerase_deHydtase.
    IPR016040. NAD(P)-bd_dom.
    [Graphical view]
    PfamiPF01370. Epimerase. 1 hit.
    [Graphical view]

    Sequencei

    Sequence statusi: Complete.

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

    Q16795-1 [UniParc]FASTAAdd to Basket

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    MAAAAQSRVV RVLSMSRSAI TAIATSVCHG PPCRQLHHAL MPHGKGGRSS    50
    VSGIVATVFG ATGFLGRYVV NHLGRMGSQV IIPYRCDKYD IMHLRPMGDL 100
    GQLLFLEWDA RDKDSIRRVV QHSNVVINLI GRDWETKNFD FEDVFVKIPQ 150
    AIAQLSKEAG VEKFIHVSHL NANIKSSSRY LRNKAVGEKV VRDAFPEAII 200
    VKPSDIFGRE DRFLNSFASM HRFGPIPLGS LGWKTVKQPV YVVDVSKGIV 250
    NAVKDPDANG KSFAFVGPSR YLLFHLVKYI FAVAHRLFLP FPLPLFAYRW 300
    VARVFEISPF EPWITRDKVE RMHITDMKLP HLPGLEDLGI QATPLELKAI 350
    EVLRRHRTYR WLSAEIEDVK PAKTVNI 377
    Length:377
    Mass (Da):42,510
    Last modified:November 1, 1997 - v2
    Checksum:i66A1CC7FCE86DD0E
    GO

    Sequence cautioni

    The sequence CAA54099.1 differs from that shown. Reason: Frameshift at position 18.

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AF050641 mRNA. Translation: AAD42055.1.
    BC009311 mRNA. Translation: AAH09311.1.
    BC015837 mRNA. Translation: AAH15837.1.
    BC111546 mRNA. Translation: AAI11547.1.
    X76665 Genomic DNA. Translation: CAA54099.1. Frameshift.
    L04490 mRNA. Translation: AAA36350.1.
    CCDSiCCDS8532.1.
    PIRiI37258.
    RefSeqiNP_004993.1. NM_005002.4.
    UniGeneiHs.75227.

    Genome annotation databases

    EnsembliENST00000266544; ENSP00000266544; ENSG00000139180.
    GeneIDi4704.
    KEGGihsa:4704.
    UCSCiuc001qnc.3. human.

    Polymorphism databases

    DMDMi2833280.

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AF050641 mRNA. Translation: AAD42055.1 .
    BC009311 mRNA. Translation: AAH09311.1 .
    BC015837 mRNA. Translation: AAH15837.1 .
    BC111546 mRNA. Translation: AAI11547.1 .
    X76665 Genomic DNA. Translation: CAA54099.1 . Frameshift.
    L04490 mRNA. Translation: AAA36350.1 .
    CCDSi CCDS8532.1.
    PIRi I37258.
    RefSeqi NP_004993.1. NM_005002.4.
    UniGenei Hs.75227.

    3D structure databases

    ProteinModelPortali Q16795.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 110784. 23 interactions.
    DIPi DIP-38526N.
    IntActi Q16795. 15 interactions.
    MINTi MINT-3033599.
    STRINGi 9606.ENSP00000266544.

    Chemistry

    ChEMBLi CHEMBL2363065.
    DrugBanki DB00157. NADH.

    PTM databases

    PhosphoSitei Q16795.

    Polymorphism databases

    DMDMi 2833280.

    Proteomic databases

    MaxQBi Q16795.
    PaxDbi Q16795.
    PRIDEi Q16795.

    Protocols and materials databases

    DNASUi 4704.
    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000266544 ; ENSP00000266544 ; ENSG00000139180 .
    GeneIDi 4704.
    KEGGi hsa:4704.
    UCSCi uc001qnc.3. human.

    Organism-specific databases

    CTDi 4704.
    GeneCardsi GC12P004714.
    HGNCi HGNC:7693. NDUFA9.
    HPAi HPA042268.
    MIMi 603834. gene.
    neXtProti NX_Q16795.
    Orphaneti 2609. Isolated NADH-CoQ reductase deficiency.
    PharmGKBi PA31499.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi COG0702.
    HOGENOMi HOG000168008.
    HOVERGENi HBG006546.
    InParanoidi Q16795.
    KOi K03953.
    OMAi IFLEWNG.
    PhylomeDBi Q16795.
    TreeFami TF105961.

    Enzyme and pathway databases

    Reactomei REACT_22393. Respiratory electron transport.

    Miscellaneous databases

    GeneWikii NDUFA9.
    GenomeRNAii 4704.
    NextBioi 18140.
    PROi Q16795.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi Q16795.
    Bgeei Q16795.
    CleanExi HS_NDUFA9.
    Genevestigatori Q16795.

    Family and domain databases

    Gene3Di 3.40.50.720. 1 hit.
    InterProi IPR001509. Epimerase_deHydtase.
    IPR016040. NAD(P)-bd_dom.
    [Graphical view ]
    Pfami PF01370. Epimerase. 1 hit.
    [Graphical view ]
    ProtoNeti Search...

    Publicationsi

    1. "39 kDa subunit of NADH-ubiquinone oxidoreductase."
      Loeffen J.L.C.M., Smeets R.J.P., Triepels R., Ruitenbeek W., Smeitink J.A.M., van den Heuvel L.
      Submitted (FEB-1998) to the EMBL/GenBank/DDBJ databases
      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: Colon, Muscle and Skeletal muscle.
    3. "Purification of CpG islands using a methylated DNA binding column."
      Cross S.H., Charlton J.A., Nan X., Bird A.P.
      Nat. Genet. 6:236-244(1994) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 1-33.
      Tissue: Blood.
    4. "Construction and evaluation of a hncDNA library of human 12p transcribed sequences derived from a somatic cell hybrid."
      Baens M., Chaffanet M., Cassiman J.-J., van den Berghe H., Marynen P.
      Genomics 16:214-218(1993) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 3-377.
      Tissue: Liver.
    5. "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.
    6. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    7. "Identification of a molecular component of the mitochondrial acetyl transferase program; a novel role for GCN5L1."
      Scott I., Webster B.R., Li J.H., Sack M.N.
      Biochem. J. 443:655-661(2012) [PubMed] [Europe PMC] [Abstract]
      Cited for: INTERACTION WITH BLOC1S1, ACETYLATION.

    Entry informationi

    Entry nameiNDUA9_HUMAN
    AccessioniPrimary (citable) accession number: Q16795
    Secondary accession number(s): Q14076, Q2NKX0
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: November 1, 1997
    Last sequence update: November 1, 1997
    Last modified: October 1, 2014
    This is version 126 of the entry and version 2 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 12
      Human chromosome 12: 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