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O95299

- NDUAA_HUMAN

UniProt

O95299 - NDUAA_HUMAN

Protein

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial

Gene

NDUFA10

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 125 (01 Oct 2014)
      Sequence version 1 (01 May 1999)
      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. ATP binding Source: InterPro
    2. NADH dehydrogenase (ubiquinone) activity Source: UniProtKB
    3. phosphotransferase activity, alcohol group as acceptor Source: InterPro

    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. response to drug Source: Ensembl
    5. small molecule metabolic process Source: Reactome

    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 10, mitochondrial
    Alternative name(s):
    Complex I-42kD
    Short name:
    CI-42kD
    NADH-ubiquinone oxidoreductase 42 kDa subunit
    Gene namesi
    Name:NDUFA10
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    ProteomesiUP000005640: Chromosome 2

    Organism-specific databases

    HGNCiHGNC:7684. NDUFA10.

    Subcellular locationi

    GO - Cellular componenti

    1. mitochondrial inner membrane Source: Reactome
    2. mitochondrial matrix Source: UniProtKB-SubCell
    3. mitochondrial respiratory chain complex I Source: UniProtKB

    Keywords - Cellular componenti

    Mitochondrion

    Pathology & Biotechi

    Organism-specific databases

    Orphaneti255241. Leigh syndrome with leukodystrophy.
    PharmGKBiPA31490.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transit peptidei1 – 3535MitochondrionBy similarityAdd
    BLAST
    Chaini36 – 355320NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrialPRO_0000019988Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei285 – 2851N6-succinyllysineBy similarity

    Proteomic databases

    MaxQBiO95299.
    PaxDbiO95299.
    PeptideAtlasiO95299.
    PRIDEiO95299.

    PTM databases

    PhosphoSiteiO95299.

    Expressioni

    Gene expression databases

    ArrayExpressiO95299.
    BgeeiO95299.
    CleanExiHS_NDUFA10.
    GenevestigatoriO95299.

    Interactioni

    Subunit structurei

    Complex I is composed of 45 different subunits. This a component of the hydrophobic protein fraction.1 Publication

    Protein-protein interaction databases

    BioGridi110785. 12 interactions.
    IntActiO95299. 5 interactions.
    MINTiMINT-4991752.
    STRINGi9606.ENSP00000252711.

    Structurei

    3D structure databases

    ProteinModelPortaliO95299.
    SMRiO95299. Positions 60-245.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Sequence similaritiesi

    Belongs to the complex I NDUFA10 subunit family.Curated

    Keywords - Domaini

    Transit peptide

    Phylogenomic databases

    eggNOGiCOG1428.
    HOGENOMiHOG000267026.
    HOVERGENiHBG002308.
    KOiK03954.
    PhylomeDBiO95299.
    TreeFamiTF314616.

    Family and domain databases

    Gene3Di3.40.50.300. 1 hit.
    InterProiIPR002624. DNK.
    IPR015828. NADH_UbQ_OxRdtase_42KD_su.
    IPR027417. P-loop_NTPase.
    [Graphical view]
    PANTHERiPTHR10513. PTHR10513. 1 hit.
    PfamiPF01712. dNK. 1 hit.
    [Graphical view]
    PIRSFiPIRSF000543. NADH_UQ_42KD. 1 hit.
    SUPFAMiSSF52540. SSF52540. 1 hit.

    Sequences (2)i

    Sequence statusi: Complete.

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

    This entry describes 2 isoformsi produced by alternative splicing. Align

    Isoform 1 (identifier: O95299-1) [UniParc]FASTAAdd to Basket

    This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

    « Hide

    MALRLLKLAA TSASARVVAA GAQRVRGIHS SVQCKLRYGM WHFLLGDKAS    50
    KRLTERSRVI TVDGNICTGK GKLAKEIAEK LGFKHFPEAG IHYPDSTTGD 100
    GKPLATDYNG NCSLEKFYDD PRSNDGNSYR LQSWLYSSRL LQYSDALEHL 150
    LTTGQGVVLE RSIFSDFVFL EAMYNQGFIR KQCVDHYNEV KSVTICDYLP 200
    PHLVIYIDVP VPEVQRRIQK KGDPHEMKIT SAYLQDIENA YKKTFLPEMS 250
    EKCEVLQYSA REAQDSKKVV EDIEYLKFDK GPWLKQDNRT LYHLRLLVQD 300
    KFEVLNYTSI PIFLPEVTIG AHQTDRVLHQ FRELPGRKYS PGYNTEVGDK 350
    WIWLK 355
    Length:355
    Mass (Da):40,751
    Last modified:May 1, 1999 - v1
    Checksum:iB5C27BC150A3E691
    GO
    Isoform 2 (identifier: O95299-2) [UniParc]FASTAAdd to Basket

    The sequence of this isoform differs from the canonical sequence as follows:
         183-183: C → CESALQTHFWTGVAGASGKLESGSSEEVLLINERGGRSKPG
         214-223: Missing.
         334-355: LPGRKYSPGYNTEVGDKWIWLK → RLDWTVCFGE...MHTTAFMFLC

    Show »
    Length:429
    Mass (Da):48,563
    Checksum:i37689EA2FA488544
    GO

    Natural variant

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti2 – 21A → G.
    Corresponds to variant rs11541494 [ dbSNP | Ensembl ].
    VAR_034149

    Alternative sequence

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Alternative sequencei183 – 1831C → CESALQTHFWTGVAGASGKL ESGSSEEVLLINERGGRSKP G in isoform 2. 1 PublicationVSP_056417
    Alternative sequencei214 – 22310Missing in isoform 2. 1 PublicationVSP_056418
    Alternative sequencei334 – 35522LPGRK…WIWLK → RLDWTVCFGEESTEVKHQGH LLSVQPGTVALTVGSWLRSC LLGLHWKLLFLFPESPMHTT AFMFLC in isoform 2. 1 PublicationVSP_056419Add
    BLAST

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AF087661 mRNA. Translation: AAD09755.1.
    AF453834 mRNA. Translation: AAL50984.1.
    AC013469 Genomic DNA. No translation available.
    AC114750 Genomic DNA. No translation available.
    AC233275 Genomic DNA. No translation available.
    BC003417 mRNA. Translation: AAH03417.1.
    CCDSiCCDS2531.1.
    PIRiJE0385.
    RefSeqiNP_004535.1. NM_004544.3.
    UniGeneiHs.277677.

    Genome annotation databases

    EnsembliENST00000252711; ENSP00000252711; ENSG00000130414.
    ENST00000307300; ENSP00000302321; ENSG00000130414.
    GeneIDi4705.
    KEGGihsa:4705.
    UCSCiuc002vyn.3. human.

    Keywords - Coding sequence diversityi

    Alternative splicing, Polymorphism

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AF087661 mRNA. Translation: AAD09755.1 .
    AF453834 mRNA. Translation: AAL50984.1 .
    AC013469 Genomic DNA. No translation available.
    AC114750 Genomic DNA. No translation available.
    AC233275 Genomic DNA. No translation available.
    BC003417 mRNA. Translation: AAH03417.1 .
    CCDSi CCDS2531.1.
    PIRi JE0385.
    RefSeqi NP_004535.1. NM_004544.3.
    UniGenei Hs.277677.

    3D structure databases

    ProteinModelPortali O95299.
    SMRi O95299. Positions 60-245.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 110785. 12 interactions.
    IntActi O95299. 5 interactions.
    MINTi MINT-4991752.
    STRINGi 9606.ENSP00000252711.

    Chemistry

    BindingDBi O95299.
    ChEMBLi CHEMBL2363065.
    DrugBanki DB00157. NADH.

    PTM databases

    PhosphoSitei O95299.

    Proteomic databases

    MaxQBi O95299.
    PaxDbi O95299.
    PeptideAtlasi O95299.
    PRIDEi O95299.

    Protocols and materials databases

    DNASUi 4705.
    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000252711 ; ENSP00000252711 ; ENSG00000130414 .
    ENST00000307300 ; ENSP00000302321 ; ENSG00000130414 .
    GeneIDi 4705.
    KEGGi hsa:4705.
    UCSCi uc002vyn.3. human.

    Organism-specific databases

    CTDi 4705.
    GeneCardsi GC02M240854.
    HGNCi HGNC:7684. NDUFA10.
    MIMi 603835. gene.
    neXtProti NX_O95299.
    Orphaneti 255241. Leigh syndrome with leukodystrophy.
    PharmGKBi PA31490.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi COG1428.
    HOGENOMi HOG000267026.
    HOVERGENi HBG002308.
    KOi K03954.
    PhylomeDBi O95299.
    TreeFami TF314616.

    Enzyme and pathway databases

    Reactomei REACT_22393. Respiratory electron transport.

    Miscellaneous databases

    ChiTaRSi NDUFA10. human.
    GeneWikii NDUFA10.
    GenomeRNAii 4705.
    NextBioi 18144.
    PROi O95299.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi O95299.
    Bgeei O95299.
    CleanExi HS_NDUFA10.
    Genevestigatori O95299.

    Family and domain databases

    Gene3Di 3.40.50.300. 1 hit.
    InterProi IPR002624. DNK.
    IPR015828. NADH_UbQ_OxRdtase_42KD_su.
    IPR027417. P-loop_NTPase.
    [Graphical view ]
    PANTHERi PTHR10513. PTHR10513. 1 hit.
    Pfami PF01712. dNK. 1 hit.
    [Graphical view ]
    PIRSFi PIRSF000543. NADH_UQ_42KD. 1 hit.
    SUPFAMi SSF52540. SSF52540. 1 hit.
    ProtoNeti Search...

    Publicationsi

    1. "cDNA of eight nuclear encoded subunits of NADH:ubiquinone oxidoreductase: human complex I cDNA characterization completed."
      Loeffen J.L.C.M., Triepels R.H., van den Heuvel L.P., Schuelke M., Buskens C.A.F., Smeets R.J.P., Trijbels J.M.F., Smeitink J.A.M.
      Biochem. Biophys. Res. Commun. 253:415-422(1998) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
    2. "Homo sapiens, NADH dehydrogenase (ubiquinone) 1 alpha subcomplex."
      Hu W., Tang L.-J., Shi Y.-W., Tian J.-Y., Jian Y.-S.
      Submitted (NOV-2001) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 2).
    3. "Generation and annotation of the DNA sequences of human chromosomes 2 and 4."
      Hillier L.W., Graves T.A., Fulton R.S., Fulton L.A., Pepin K.H., Minx P., Wagner-McPherson C., Layman D., Wylie K., Sekhon M., Becker M.C., Fewell G.A., Delehaunty K.D., Miner T.L., Nash W.E., Kremitzki C., Oddy L., Du H.
      , Sun H., Bradshaw-Cordum H., Ali J., Carter J., Cordes M., Harris A., Isak A., van Brunt A., Nguyen C., Du F., Courtney L., Kalicki J., Ozersky P., Abbott S., Armstrong J., Belter E.A., Caruso L., Cedroni M., Cotton M., Davidson T., Desai A., Elliott G., Erb T., Fronick C., Gaige T., Haakenson W., Haglund K., Holmes A., Harkins R., Kim K., Kruchowski S.S., Strong C.M., Grewal N., Goyea E., Hou S., Levy A., Martinka S., Mead K., McLellan M.D., Meyer R., Randall-Maher J., Tomlinson C., Dauphin-Kohlberg S., Kozlowicz-Reilly A., Shah N., Swearengen-Shahid S., Snider J., Strong J.T., Thompson J., Yoakum M., Leonard S., Pearman C., Trani L., Radionenko M., Waligorski J.E., Wang C., Rock S.M., Tin-Wollam A.-M., Maupin R., Latreille P., Wendl M.C., Yang S.-P., Pohl C., Wallis J.W., Spieth J., Bieri T.A., Berkowicz N., Nelson J.O., Osborne J., Ding L., Meyer R., Sabo A., Shotland Y., Sinha P., Wohldmann P.E., Cook L.L., Hickenbotham M.T., Eldred J., Williams D., Jones T.A., She X., Ciccarelli F.D., Izaurralde E., Taylor J., Schmutz J., Myers R.M., Cox D.R., Huang X., McPherson J.D., Mardis E.R., Clifton S.W., Warren W.C., Chinwalla A.T., Eddy S.R., Marra M.A., Ovcharenko I., Furey T.S., Miller W., Eichler E.E., Bork P., Suyama M., Torrents D., Waterston R.H., Wilson R.K.
      Nature 434:724-731(2005) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
    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] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
      Tissue: Placenta.
    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].

    Entry informationi

    Entry nameiNDUAA_HUMAN
    AccessioniPrimary (citable) accession number: O95299
    Secondary accession number(s): Q8WXC9
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: July 15, 1999
    Last sequence update: May 1, 1999
    Last modified: October 1, 2014
    This is version 125 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 2
      Human chromosome 2: entries, gene names and cross-references to MIM
    2. Human entries with polymorphisms or disease mutations
      List of human entries with polymorphisms or disease mutations
    3. Human polymorphisms and disease mutations
      Index of human polymorphisms and disease mutations
    4. MIM cross-references
      Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
    5. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3