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Q9NZJ6

- COQ3_HUMAN

UniProt

Q9NZJ6 - COQ3_HUMAN

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Protein
Hexaprenyldihydroxybenzoate methyltransferase, mitochondrial
Gene
COQ3, UG0215E05
Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5 - Experimental evidence at protein leveli

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. O-methyltransferase activity Source: ProtInc
  4. hexaprenyldihydroxybenzoate methyltransferase activity Source: UniProtKB-EC

GO - Biological processi

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

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
Complete GO annotation...

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 – ?Mitochondrion Reviewed prediction

Amino acid modifications

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

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

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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.

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 conflict

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

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

« Hide 'large scale' publications
  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: September 3, 2014
This is version 129 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

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