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Q9NZJ6

- COQ3_HUMAN

UniProt

Q9NZJ6 - COQ3_HUMAN

Protein

Hexaprenyldihydroxybenzoate methyltransferase, mitochondrial

Gene

COQ3

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 130 (01 Oct 2014)
      Sequence version 3 (30 Nov 2010)
      Previous versions | rss
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    Functioni

    Catalytic activityi

    S-adenosyl-L-methionine + 3,4-dihydroxy-5-all-trans-polyprenylbenzoate = S-adenosyl-L-homocysteine + 3-methoxy-4-hydroxy-5-all-trans-polyprenylbenzoate.1 Publication
    S-adenosyl-L-methionine + 3-demethylubiquinone-n = S-adenosyl-L-homocysteine + ubiquinone-n.1 Publication
    S-adenosyl-L-methionine + 3-(all-trans-polyprenyl)benzene-1,2-diol = S-adenosyl-L-homocysteine + 2-methoxy-6-(all-trans-polyprenyl)phenol.1 Publication

    Pathwayi

    GO - Molecular functioni

    1. 2-polyprenyl-6-methoxy-1,4-benzoquinone methyltransferase activity Source: InterPro
    2. 3-demethylubiquinone-9 3-O-methyltransferase activity Source: UniProtKB-EC
    3. hexaprenyldihydroxybenzoate methyltransferase activity Source: UniProtKB-EC
    4. O-methyltransferase activity Source: ProtInc

    GO - Biological processi

    1. glycerol metabolic process Source: UniProtKB
    2. small molecule metabolic process Source: Reactome
    3. ubiquinone biosynthetic process Source: UniProtKB

    Keywords - Molecular functioni

    Methyltransferase, Transferase

    Keywords - Biological processi

    Ubiquinone biosynthesis

    Keywords - Ligandi

    S-adenosyl-L-methionine

    Enzyme and pathway databases

    BioCyciMetaCyc:HS05632-MONOMER.
    ReactomeiREACT_160111. Ubiquinol biosynthesis.
    UniPathwayiUPA00232.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Hexaprenyldihydroxybenzoate methyltransferase, mitochondrial (EC:2.1.1.114)
    Alternative name(s):
    2-polyprenyl-6-hydroxyphenol methylase (EC:2.1.1.222)
    3,4-dihydroxy-5-hexaprenylbenzoate methyltransferase
    Short name:
    DHHB methyltransferase
    Short name:
    DHHB-MT
    Short name:
    DHHB-MTase
    3-demethylubiquinone-10 3-methyltransferase (EC:2.1.1.64)
    Dihydroxyhexaprenylbenzoate methyltransferase
    Gene namesi
    Name:COQ3
    ORF Names:UG0215E05
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    ProteomesiUP000005640: Chromosome 6

    Organism-specific databases

    HGNCiHGNC:18175. COQ3.

    Subcellular locationi

    GO - Cellular componenti

    1. mitochondrial matrix Source: Reactome

    Keywords - Cellular componenti

    Mitochondrion

    Pathology & Biotechi

    Organism-specific databases

    PharmGKBiPA134934287.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Chaini? – 369Hexaprenyldihydroxybenzoate methyltransferase, mitochondrialPRO_0000035926
    Transit peptidei1 – ?MitochondrionSequence Analysis

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei143 – 1431N6-acetyllysineBy similarity
    Modified residuei149 – 1491N6-acetyllysineBy similarity
    Modified residuei196 – 1961N6-acetyllysineBy similarity

    Keywords - PTMi

    Acetylation

    Proteomic databases

    MaxQBiQ9NZJ6.
    PaxDbiQ9NZJ6.
    PRIDEiQ9NZJ6.

    PTM databases

    PhosphoSiteiQ9NZJ6.

    Expressioni

    Gene expression databases

    ArrayExpressiQ9NZJ6.
    BgeeiQ9NZJ6.
    CleanExiHS_COQ3.
    GenevestigatoriQ9NZJ6.

    Organism-specific databases

    HPAiHPA031273.
    HPA031274.

    Interactioni

    Protein-protein interaction databases

    BioGridi119731. 1 interaction.
    STRINGi9606.ENSP00000254759.

    Structurei

    3D structure databases

    ProteinModelPortaliQ9NZJ6.
    SMRiQ9NZJ6. Positions 149-299.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Sequence similaritiesi

    Keywords - Domaini

    Transit peptide

    Phylogenomic databases

    eggNOGiCOG2227.
    HOVERGENiHBG051078.
    InParanoidiQ9NZJ6.
    KOiK00591.
    OMAiNYMVHTH.
    OrthoDBiEOG7FJH1X.
    PhylomeDBiQ9NZJ6.
    TreeFamiTF314553.

    Family and domain databases

    Gene3Di3.40.50.150. 1 hit.
    InterProiIPR013216. Methyltransf_11.
    IPR029063. SAM-dependent_MTases-like.
    IPR010233. UbiG_MeTrfase.
    [Graphical view]
    PANTHERiPTHR23134. PTHR23134. 1 hit.
    PfamiPF08241. Methyltransf_11. 1 hit.
    [Graphical view]
    SUPFAMiSSF53335. SSF53335. 1 hit.
    TIGRFAMsiTIGR01983. UbiG. 1 hit.

    Sequencei

    Sequence statusi: Complete.

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

    Q9NZJ6-1 [UniParc]FASTAAdd to Basket

    « Hide

    MWSGRKLGSS GGWFLRVLGP GGCNTKAARP LISSAVYVKN QLSGTLQIKP    50
    GVFNEYRTIW FKSYRTIFSC LNRIKSFRYP WARLYSTSQT TVDSGEVKTF 100
    LALAHKWWDE QGVYAPLHSM NDLRVPFIRD NLLKTIPNHQ PGKPLLGMKI 150
    LDVGCGGGLL TEPLGRLGAS VIGIDPVDEN IKTAQCHKSF DPVLDKRIEY 200
    RVCSLEEIVE ETAETFDAVV ASEVVEHVID LETFLQCCCQ VLKPGGSLFI 250
    TTINKTQLSY ALGIVFSEQI ASIVPKGTHT WEKFVSPETL ESILESNGLS 300
    VQTVVGMLYN PFSGYWHWSE NTSLNYAAYA VKSRVQEHPA SAEFVLKGET 350
    EELQANACTN PAVHEKLKK 369
    Length:369
    Mass (Da):41,054
    Last modified:November 30, 2010 - v3
    Checksum:iB257A099D6C06A2D
    GO

    Sequence cautioni

    The sequence AAF66826.1 differs from that shown. Reason: Frameshift at positions 16, 18, 22, 26, 44, 49, 60, 363 and 364.

    Experimental Info

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sequence conflicti21 – 211G → V in CAB66660. (PubMed:11230166)Curated
    Sequence conflicti180 – 1801N → D in CAB66660. (PubMed:11230166)Curated
    Sequence conflicti287 – 2893PET → LEP in AAN76515. 1 PublicationCurated
    Sequence conflicti321 – 3211N → D in AAN76515. 1 PublicationCurated

    Natural variant

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti134 – 1341K → E.2 Publications
    Corresponds to variant rs11548336 [ dbSNP | Ensembl ].
    VAR_061925
    Natural varianti272 – 2721S → G.4 Publications
    Corresponds to variant rs6925344 [ dbSNP | Ensembl ].
    VAR_020789
    Natural varianti329 – 3291Y → H.5 Publications
    Corresponds to variant rs4144164 [ dbSNP | Ensembl ].
    VAR_020790

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AF351615 mRNA. Translation: AAN76515.1.
    AL136726 mRNA. Translation: CAB66660.1.
    AK056955 mRNA. Translation: BAG51832.1.
    AL513550 Genomic DNA. Translation: CAI17193.1.
    BC015634 mRNA. Translation: AAH15634.2.
    BC063463 mRNA. Translation: AAH63463.1.
    AF193016 mRNA. Translation: AAF66826.1. Frameshift.
    CCDSiCCDS5042.1.
    RefSeqiNP_059117.3. NM_017421.3.
    UniGeneiHs.713623.

    Genome annotation databases

    EnsembliENST00000254759; ENSP00000254759; ENSG00000132423.
    GeneIDi51805.
    KEGGihsa:51805.
    UCSCiuc003ppk.3. human.

    Polymorphism databases

    DMDMi313104241.

    Keywords - Coding sequence diversityi

    Polymorphism

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AF351615 mRNA. Translation: AAN76515.1 .
    AL136726 mRNA. Translation: CAB66660.1 .
    AK056955 mRNA. Translation: BAG51832.1 .
    AL513550 Genomic DNA. Translation: CAI17193.1 .
    BC015634 mRNA. Translation: AAH15634.2 .
    BC063463 mRNA. Translation: AAH63463.1 .
    AF193016 mRNA. Translation: AAF66826.1 . Frameshift.
    CCDSi CCDS5042.1.
    RefSeqi NP_059117.3. NM_017421.3.
    UniGenei Hs.713623.

    3D structure databases

    ProteinModelPortali Q9NZJ6.
    SMRi Q9NZJ6. Positions 149-299.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 119731. 1 interaction.
    STRINGi 9606.ENSP00000254759.

    PTM databases

    PhosphoSitei Q9NZJ6.

    Polymorphism databases

    DMDMi 313104241.

    Proteomic databases

    MaxQBi Q9NZJ6.
    PaxDbi Q9NZJ6.
    PRIDEi Q9NZJ6.

    Protocols and materials databases

    DNASUi 51805.
    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000254759 ; ENSP00000254759 ; ENSG00000132423 .
    GeneIDi 51805.
    KEGGi hsa:51805.
    UCSCi uc003ppk.3. human.

    Organism-specific databases

    CTDi 51805.
    GeneCardsi GC06M099817.
    H-InvDB HIX0200854.
    HGNCi HGNC:18175. COQ3.
    HPAi HPA031273.
    HPA031274.
    MIMi 605196. gene.
    neXtProti NX_Q9NZJ6.
    PharmGKBi PA134934287.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi COG2227.
    HOVERGENi HBG051078.
    InParanoidi Q9NZJ6.
    KOi K00591.
    OMAi NYMVHTH.
    OrthoDBi EOG7FJH1X.
    PhylomeDBi Q9NZJ6.
    TreeFami TF314553.

    Enzyme and pathway databases

    UniPathwayi UPA00232 .
    BioCyci MetaCyc:HS05632-MONOMER.
    Reactomei REACT_160111. Ubiquinol biosynthesis.

    Miscellaneous databases

    GenomeRNAii 51805.
    NextBioi 55924.
    PROi Q9NZJ6.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi Q9NZJ6.
    Bgeei Q9NZJ6.
    CleanExi HS_COQ3.
    Genevestigatori Q9NZJ6.

    Family and domain databases

    Gene3Di 3.40.50.150. 1 hit.
    InterProi IPR013216. Methyltransf_11.
    IPR029063. SAM-dependent_MTases-like.
    IPR010233. UbiG_MeTrfase.
    [Graphical view ]
    PANTHERi PTHR23134. PTHR23134. 1 hit.
    Pfami PF08241. Methyltransf_11. 1 hit.
    [Graphical view ]
    SUPFAMi SSF53335. SSF53335. 1 hit.
    TIGRFAMsi TIGR01983. UbiG. 1 hit.
    ProtoNeti Search...

    Publicationsi

    1. "Isolation of full-length cDNA clones from human fetal brain cDNA library."
      Xie Y., Mao Y.
      Submitted (FEB-2001) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA], VARIANTS GLU-134; GLY-272 AND HIS-329.
      Tissue: Fetal brain.
    2. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA], VARIANT HIS-329.
      Tissue: Kidney.
    3. "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], VARIANTS GLY-272 AND HIS-329.
      Tissue: Skeletal muscle.
    4. "The DNA sequence and analysis of human chromosome 6."
      Mungall A.J., Palmer S.A., Sims S.K., Edwards C.A., Ashurst J.L., Wilming L., Jones M.C., Horton R., Hunt S.E., Scott C.E., Gilbert J.G.R., Clamp M.E., Bethel G., Milne S., Ainscough R., Almeida J.P., Ambrose K.D., Andrews T.D.
      , Ashwell R.I.S., Babbage A.K., Bagguley C.L., Bailey J., Banerjee R., Barker D.J., Barlow K.F., Bates K., Beare D.M., Beasley H., Beasley O., Bird C.P., Blakey S.E., Bray-Allen S., Brook J., Brown A.J., Brown J.Y., Burford D.C., Burrill W., Burton J., Carder C., Carter N.P., Chapman J.C., Clark S.Y., Clark G., Clee C.M., Clegg S., Cobley V., Collier R.E., Collins J.E., Colman L.K., Corby N.R., Coville G.J., Culley K.M., Dhami P., Davies J., Dunn M., Earthrowl M.E., Ellington A.E., Evans K.A., Faulkner L., Francis M.D., Frankish A., Frankland J., French L., Garner P., Garnett J., Ghori M.J., Gilby L.M., Gillson C.J., Glithero R.J., Grafham D.V., Grant M., Gribble S., Griffiths C., Griffiths M.N.D., Hall R., Halls K.S., Hammond S., Harley J.L., Hart E.A., Heath P.D., Heathcott R., Holmes S.J., Howden P.J., Howe K.L., Howell G.R., Huckle E., Humphray S.J., Humphries M.D., Hunt A.R., Johnson C.M., Joy A.A., Kay M., Keenan S.J., Kimberley A.M., King A., Laird G.K., Langford C., Lawlor S., Leongamornlert D.A., Leversha M., Lloyd C.R., Lloyd D.M., Loveland J.E., Lovell J., Martin S., Mashreghi-Mohammadi M., Maslen G.L., Matthews L., McCann O.T., McLaren S.J., McLay K., McMurray A., Moore M.J.F., Mullikin J.C., Niblett D., Nickerson T., Novik K.L., Oliver K., Overton-Larty E.K., Parker A., Patel R., Pearce A.V., Peck A.I., Phillimore B.J.C.T., Phillips S., Plumb R.W., Porter K.M., Ramsey Y., Ranby S.A., Rice C.M., Ross M.T., Searle S.M., Sehra H.K., Sheridan E., Skuce C.D., Smith S., Smith M., Spraggon L., Squares S.L., Steward C.A., Sycamore N., Tamlyn-Hall G., Tester J., Theaker A.J., Thomas D.W., Thorpe A., Tracey A., Tromans A., Tubby B., Wall M., Wallis J.M., West A.P., White S.S., Whitehead S.L., Whittaker H., Wild A., Willey D.J., Wilmer T.E., Wood J.M., Wray P.W., Wyatt J.C., Young L., Younger R.M., Bentley D.R., Coulson A., Durbin R.M., Hubbard T., Sulston J.E., Dunham I., Rogers J., Beck S.
      Nature 425:805-811(2003) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
    5. "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], VARIANTS GLU-134; GLY-272 AND HIS-329.
      Tissue: Brain and Skin.
    6. "Isolation and functional expression of human COQ3, a gene encoding a methyltransferase required for ubiquinone biosynthesis."
      Jonassen T., Clarke C.F.
      J. Biol. Chem. 275:12381-12387(2000) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 11-369, CATALYTIC ACTIVITY, VARIANTS GLY-272 AND HIS-329.
    7. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].

    Entry informationi

    Entry nameiCOQ3_HUMAN
    AccessioniPrimary (citable) accession number: Q9NZJ6
    Secondary accession number(s): B3KPX0
    , Q5T061, Q6P4F0, Q8IXG6, Q96BG1, Q9H0N1
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: November 2, 2001
    Last sequence update: November 30, 2010
    Last modified: October 1, 2014
    This is version 130 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

    Keywords - Technical termi

    Complete proteome, Reference proteome

    Documents

    1. Human chromosome 6
      Human chromosome 6: 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. PATHWAY comments
      Index of metabolic and biosynthesis pathways
    6. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3