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P56556

- NDUA6_HUMAN

UniProt

P56556 - NDUA6_HUMAN

Protein

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6

Gene

NDUFA6

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 3 (15 Jun 2010)
      Previous versions | rss
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    Functioni

    Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed to be not 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. NADH dehydrogenase (ubiquinone) activity Source: UniProtKB

    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 oxidative stress Source: UniProtKB
    5. 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] 1 alpha subcomplex subunit 6
    Alternative name(s):
    Complex I-B14
    Short name:
    CI-B14
    LYR motif-containing protein 6
    NADH-ubiquinone oxidoreductase B14 subunit
    Gene namesi
    Name:NDUFA6
    Synonyms:LYRM6, NADHB14
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    ProteomesiUP000005640: Chromosome 22

    Organism-specific databases

    HGNCiHGNC:7690. NDUFA6.

    Subcellular locationi

    GO - Cellular componenti

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

    Keywords - Cellular componenti

    Membrane, Mitochondrion, Mitochondrion inner membrane

    Pathology & Biotechi

    Organism-specific databases

    PharmGKBiPA31496.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Chaini1 – 154154NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6PRO_0000174302Add
    BLAST

    Proteomic databases

    MaxQBiP56556.
    PaxDbiP56556.
    PRIDEiP56556.

    PTM databases

    PhosphoSiteiP56556.

    Expressioni

    Gene expression databases

    BgeeiP56556.
    CleanExiHS_NDUFA6.
    GenevestigatoriP56556.

    Organism-specific databases

    HPAiHPA056934.

    Interactioni

    Subunit structurei

    Mammalian complex I is composed of 45 different subunits.1 Publication

    Protein-protein interaction databases

    BioGridi110780. 5 interactions.
    IntActiP56556. 5 interactions.
    MINTiMINT-1381261.
    STRINGi9606.ENSP00000330937.

    Structurei

    3D structure databases

    ProteinModelPortaliP56556.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Sequence similaritiesi

    Belongs to the complex I LYR family.Curated

    Phylogenomic databases

    eggNOGiNOG283416.
    HOGENOMiHOG000200993.
    HOVERGENiHBG002032.
    InParanoidiP56556.
    KOiK03950.
    OMAiPIFSRDL.
    PhylomeDBiP56556.
    TreeFamiTF105625.

    Family and domain databases

    InterProiIPR008011. Complex1_LYR.
    IPR016488. NADH_Ub_cplx-1_asu_su-6.
    [Graphical view]
    PfamiPF05347. Complex1_LYR. 1 hit.
    [Graphical view]
    PIRSFiPIRSF006643. NDUA6. 1 hit.

    Sequencei

    Sequence statusi: Complete.

    P56556-1 [UniParc]FASTAAdd to Basket

    « Hide

    MGKDIRPRSA RAACKGVGLW SGCFGKMAGS GVRQATSTAS TFVKPIFSRD    50
    MNEAKRRVRE LYRAWYREVP NTVHQFQLDI TVKMGRDKVR EMFMKNAHVT 100
    DPRVVDLLVI KGKIELEETI KVWKQRTHVM RFFHETEAPR PKDFLSKFYV 150
    GHDP 154
    Length:154
    Mass (Da):17,871
    Last modified:June 15, 2010 - v3
    Checksum:iC3423B7B75A6336A
    GO

    Sequence cautioni

    The sequence AAC04267.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.
    The sequence BAF84563.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.
    The sequence BAG38151.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.
    The sequence EAW60489.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.

    Experimental Info

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sequence conflicti32 – 321V → F in CAG32995. 1 PublicationCurated

    Natural variant

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti35 – 351A → V.1 Publication
    Corresponds to variant rs1801311 [ dbSNP | Ensembl ].
    VAR_014483

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    CR456529 mRNA. Translation: CAG30415.1.
    AL021878 Genomic DNA. No translation available.
    CH471095 Genomic DNA. Translation: EAW60489.1. Different initiation.
    AK291874 mRNA. Translation: BAF84563.1. Different initiation.
    AK316562 mRNA. Translation: BAG38151.1. Different initiation.
    AF047182 mRNA. Translation: AAC04267.1. Different initiation.
    CR456714 mRNA. Translation: CAG32995.1.
    CR541997 mRNA. Translation: CAG46794.1.
    BC002772 mRNA. Translation: AAH02772.1.
    CCDSiCCDS33656.1.
    PIRiJC5821.
    RefSeqiNP_002481.2. NM_002490.3.
    UniGeneiHs.274416.

    Genome annotation databases

    EnsembliENST00000498737; ENSP00000418842; ENSG00000184983.
    ENST00000602404; ENSP00000473461; ENSG00000184983.
    ENST00000605916; ENSP00000475402; ENSG00000272765.
    ENST00000607299; ENSP00000476045; ENSG00000273397.
    GeneIDi4700.
    KEGGihsa:4700.
    UCSCiuc003bcb.3. human.

    Polymorphism databases

    DMDMi298286909.

    Keywords - Coding sequence diversityi

    Polymorphism

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    CR456529 mRNA. Translation: CAG30415.1 .
    AL021878 Genomic DNA. No translation available.
    CH471095 Genomic DNA. Translation: EAW60489.1 . Different initiation.
    AK291874 mRNA. Translation: BAF84563.1 . Different initiation.
    AK316562 mRNA. Translation: BAG38151.1 . Different initiation.
    AF047182 mRNA. Translation: AAC04267.1 . Different initiation.
    CR456714 mRNA. Translation: CAG32995.1 .
    CR541997 mRNA. Translation: CAG46794.1 .
    BC002772 mRNA. Translation: AAH02772.1 .
    CCDSi CCDS33656.1.
    PIRi JC5821.
    RefSeqi NP_002481.2. NM_002490.3.
    UniGenei Hs.274416.

    3D structure databases

    ProteinModelPortali P56556.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 110780. 5 interactions.
    IntActi P56556. 5 interactions.
    MINTi MINT-1381261.
    STRINGi 9606.ENSP00000330937.

    Chemistry

    BindingDBi P56556.
    ChEMBLi CHEMBL2363065.
    DrugBanki DB00157. NADH.

    PTM databases

    PhosphoSitei P56556.

    Polymorphism databases

    DMDMi 298286909.

    Proteomic databases

    MaxQBi P56556.
    PaxDbi P56556.
    PRIDEi P56556.

    Protocols and materials databases

    DNASUi 4700.
    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000498737 ; ENSP00000418842 ; ENSG00000184983 .
    ENST00000602404 ; ENSP00000473461 ; ENSG00000184983 .
    ENST00000605916 ; ENSP00000475402 ; ENSG00000272765 .
    ENST00000607299 ; ENSP00000476045 ; ENSG00000273397 .
    GeneIDi 4700.
    KEGGi hsa:4700.
    UCSCi uc003bcb.3. human.

    Organism-specific databases

    CTDi 4700.
    GeneCardsi GC22M042481.
    HGNCi HGNC:7690. NDUFA6.
    HPAi HPA056934.
    MIMi 602138. gene.
    neXtProti NX_P56556.
    PharmGKBi PA31496.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi NOG283416.
    HOGENOMi HOG000200993.
    HOVERGENi HBG002032.
    InParanoidi P56556.
    KOi K03950.
    OMAi PIFSRDL.
    PhylomeDBi P56556.
    TreeFami TF105625.

    Enzyme and pathway databases

    Reactomei REACT_22393. Respiratory electron transport.

    Miscellaneous databases

    ChiTaRSi NDUFA6. human.
    GeneWikii NDUFA6.
    GenomeRNAii 4700.
    NextBioi 18124.
    PROi P56556.
    SOURCEi Search...

    Gene expression databases

    Bgeei P56556.
    CleanExi HS_NDUFA6.
    Genevestigatori P56556.

    Family and domain databases

    InterProi IPR008011. Complex1_LYR.
    IPR016488. NADH_Ub_cplx-1_asu_su-6.
    [Graphical view ]
    Pfami PF05347. Complex1_LYR. 1 hit.
    [Graphical view ]
    PIRSFi PIRSF006643. NDUA6. 1 hit.
    ProtoNeti Search...

    Publicationsi

    1. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    2. "The DNA sequence of human chromosome 22."
      Dunham I., Hunt A.R., Collins J.E., Bruskiewich R., Beare D.M., Clamp M., Smink L.J., Ainscough R., Almeida J.P., Babbage A.K., Bagguley C., Bailey J., Barlow K.F., Bates K.N., Beasley O.P., Bird C.P., Blakey S.E., Bridgeman A.M.
      , Buck D., Burgess J., Burrill W.D., Burton J., Carder C., Carter N.P., Chen Y., Clark G., Clegg S.M., Cobley V.E., Cole C.G., Collier R.E., Connor R., Conroy D., Corby N.R., Coville G.J., Cox A.V., Davis J., Dawson E., Dhami P.D., Dockree C., Dodsworth S.J., Durbin R.M., Ellington A.G., Evans K.L., Fey J.M., Fleming K., French L., Garner A.A., Gilbert J.G.R., Goward M.E., Grafham D.V., Griffiths M.N.D., Hall C., Hall R.E., Hall-Tamlyn G., Heathcott R.W., Ho S., Holmes S., Hunt S.E., Jones M.C., Kershaw J., Kimberley A.M., King A., Laird G.K., Langford C.F., Leversha M.A., Lloyd C., Lloyd D.M., Martyn I.D., Mashreghi-Mohammadi M., Matthews L.H., Mccann O.T., Mcclay J., Mclaren S., McMurray A.A., Milne S.A., Mortimore B.J., Odell C.N., Pavitt R., Pearce A.V., Pearson D., Phillimore B.J.C.T., Phillips S.H., Plumb R.W., Ramsay H., Ramsey Y., Rogers L., Ross M.T., Scott C.E., Sehra H.K., Skuce C.D., Smalley S., Smith M.L., Soderlund C., Spragon L., Steward C.A., Sulston J.E., Swann R.M., Vaudin M., Wall M., Wallis J.M., Whiteley M.N., Willey D.L., Williams L., Williams S.A., Williamson H., Wilmer T.E., Wilming L., Wright C.L., Hubbard T., Bentley D.R., Beck S., Rogers J., Shimizu N., Minoshima S., Kawasaki K., Sasaki T., Asakawa S., Kudoh J., Shintani A., Shibuya K., Yoshizaki Y., Aoki N., Mitsuyama S., Roe B.A., Chen F., Chu L., Crabtree J., Deschamps S., Do A., Do T., Dorman A., Fang F., Fu Y., Hu P., Hua A., Kenton S., Lai H., Lao H.I., Lewis J., Lewis S., Lin S.-P., Loh P., Malaj E., Nguyen T., Pan H., Phan S., Qi S., Qian Y., Ray L., Ren Q., Shaull S., Sloan D., Song L., Wang Q., Wang Y., Wang Z., White J., Willingham D., Wu H., Yao Z., Zhan M., Zhang G., Chissoe S., Murray J., Miller N., Minx P., Fulton R., Johnson D., Bemis G., Bentley D., Bradshaw H., Bourne S., Cordes M., Du Z., Fulton L., Goela D., Graves T., Hawkins J., Hinds K., Kemp K., Latreille P., Layman D., Ozersky P., Rohlfing T., Scheet P., Walker C., Wamsley A., Wohldmann P., Pepin K., Nelson J., Korf I., Bedell J.A., Hillier L.W., Mardis E., Waterston R., Wilson R., Emanuel B.S., Shaikh T., Kurahashi H., Saitta S., Budarf M.L., McDermid H.E., Johnson A., Wong A.C.C., Morrow B.E., Edelmann L., Kim U.J., Shizuya H., Simon M.I., Dumanski J.P., Peyrard M., Kedra D., Seroussi E., Fransson I., Tapia I., Bruder C.E., O'Brien K.P., Wilkinson P., Bodenteich A., Hartman K., Hu X., Khan A.S., Lane L., Tilahun Y., Wright H.
      Nature 402:489-495(1999) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
    3. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
    4. "Complete sequencing and characterization of 21,243 full-length human cDNAs."
      Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S.
      , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.
      Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 21-154, VARIANT VAL-35.
      Tissue: Skeletal muscle.
    5. "Identification and primary structure of five human NADH-ubiquinone oxidoreductase subunits."
      Ton C., Hwang D.M., Dempsey A.A., Liew C.-C.
      Biochem. Biophys. Res. Commun. 241:589-594(1997) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 26-154.
      Tissue: Heart.
    6. "Cloning of human full open reading frames in Gateway(TM) system entry vector (pDONR201)."
      Ebert L., Schick M., Neubert P., Schatten R., Henze S., Korn B.
      Submitted (JUN-2004) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 27-154.
    7. "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] OF 27-154.
      Tissue: Lymph.
    8. "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.
    9. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].

    Entry informationi

    Entry nameiNDUA6_HUMAN
    AccessioniPrimary (citable) accession number: P56556
    Secondary accession number(s): B2RE54
    , O43675, Q6FGW0, Q6IBT8, Q6IC39
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: July 15, 1998
    Last sequence update: June 15, 2010
    Last modified: October 1, 2014
    This is version 125 of the entry and version 3 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

    Caution

    It is uncertain whether Met-1 or Met-27 is the initiator.Curated

    Keywords - Technical termi

    Complete proteome, Reference proteome

    Documents

    1. Human chromosome 22
      Human chromosome 22: 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