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Protein

5-demethoxyubiquinone hydroxylase, mitochondrial

Gene

COQ7

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Catalyzes the hydroxylation of 2-polyprenyl-3-methyl-6-methoxy-1,4-benzoquinol (DMQH2) during ubiquinone biosynthesis. Has also a structural role in the COQ enzyme complex, stabilizing other COQ polypeptides. Involved in lifespan determination in a ubiquinone-independent manner.UniRule annotation

Cofactori

Fe cationUniRule annotationNote: Binds 2 iron ions per subunit.UniRule annotation

Pathway:iubiquinone biosynthesis

This protein is involved in the pathway ubiquinone biosynthesis, which is part of Cofactor biosynthesis.UniRule annotation
View all proteins of this organism that are known to be involved in the pathway ubiquinone biosynthesis and in Cofactor biosynthesis.

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Metal bindingi60 – 601Iron 1UniRule annotation
Metal bindingi90 – 901Iron 1UniRule annotation
Metal bindingi90 – 901Iron 2UniRule annotation
Metal bindingi93 – 931Iron 1UniRule annotation
Metal bindingi142 – 1421Iron 2UniRule annotation
Metal bindingi178 – 1781Iron 1UniRule annotation
Metal bindingi178 – 1781Iron 2UniRule annotation
Metal bindingi181 – 1811Iron 2UniRule annotation

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Monooxygenase, Oxidoreductase

Keywords - Biological processi

Ubiquinone biosynthesis

Keywords - Ligandi

Iron, Metal-binding

Enzyme and pathway databases

BioCyciMetaCyc:ENSG00000167186-MONOMER.
ReactomeiREACT_160111. Ubiquinol biosynthesis.
UniPathwayiUPA00232.

Names & Taxonomyi

Protein namesi
Recommended name:
5-demethoxyubiquinone hydroxylase, mitochondrialUniRule annotation (EC:1.14.13.-UniRule annotation)
Short name:
DMQ hydroxylaseUniRule annotation
Alternative name(s):
Timing protein clk-1 homologUniRule annotation
Ubiquinone biosynthesis monooxygenase COQ7UniRule annotation
Gene namesi
Name:COQ7UniRule annotation
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640 Componenti: Chromosome 16

Organism-specific databases

HGNCiHGNC:2244. COQ7.

Subcellular locationi

  • Mitochondrion inner membrane UniRule annotation; Peripheral membrane protein UniRule annotation; Matrix side UniRule annotation

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA26761.

Polymorphism and mutation databases

BioMutaiCOQ7.
DMDMi311033465.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 3535MitochondrionSequence AnalysisAdd
BLAST
Chaini36 – 2171825-demethoxyubiquinone hydroxylase, mitochondrialPRO_0000079251Add
BLAST

Proteomic databases

MaxQBiQ99807.
PaxDbiQ99807.
PRIDEiQ99807.

PTM databases

PhosphoSiteiQ99807.

Expressioni

Tissue specificityi

Expressed dominantly in heart and skeletal muscle.

Gene expression databases

BgeeiQ99807.
CleanExiHS_COQ7.
ExpressionAtlasiQ99807. baseline and differential.
GenevisibleiQ99807. HS.

Organism-specific databases

HPAiHPA067252.

Interactioni

Subunit structurei

Component of a multi-subunit COQ enzyme complex, composed of at least COQ3, COQ4, COQ5, COQ6, COQ7 and COQ9 (By similarity). Interacts with ADCK4 and COQ6 (PubMed:24270420). Interacts with COQ9 (PubMed:25339443).UniRule annotation2 Publications

Protein-protein interaction databases

STRINGi9606.ENSP00000322316.

Structurei

3D structure databases

ProteinModelPortaliQ99807.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Repeati48 – 129821Add
BLAST
Repeati130 – 217882Add
BLAST

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni48 – 2171702 X approximate tandem repeatsAdd
BLAST

Compositional bias

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Compositional biasi4 – 96Poly-Ala

Sequence similaritiesi

Belongs to the COQ7 family.UniRule annotation

Keywords - Domaini

Repeat, Transit peptide

Phylogenomic databases

eggNOGiCOG2941.
GeneTreeiENSGT00390000014520.
HOGENOMiHOG000184972.
HOVERGENiHBG002214.
InParanoidiQ99807.
KOiK06134.
OMAiEGHEQVH.
OrthoDBiEOG73BVF0.
PhylomeDBiQ99807.
TreeFamiTF314559.

Family and domain databases

Gene3Di1.20.1260.10. 1 hit.
HAMAPiMF_01658. COQ7.
InterProiIPR009078. Ferritin-like_SF.
IPR012347. Ferritin-rel.
IPR011566. Ubq_synth_Coq7.
[Graphical view]
PANTHERiPTHR11237. PTHR11237. 1 hit.
PfamiPF03232. COQ7. 1 hit.
[Graphical view]
SUPFAMiSSF47240. SSF47240. 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. AlignAdd to basket

Isoform 1 (identifier: Q99807-1) [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

        10         20         30         40         50
MSCAGAAAAP RLWRLRPGAR RSLSAYGRRT SVRFRSSGMT LDNISRAAVD
60 70 80 90 100
RIIRVDHAGE YGANRIYAGQ MAVLGRTSVG PVIQKMWDQE KDHLKKFNEL
110 120 130 140 150
MVTFRVRPTV LMPLWNVLGF ALGAGTALLG KEGAMACTVA VEESIAHHYN
160 170 180 190 200
NQIRTLMEED PEKYEELLQL IKKFRDEELE HHDIGLDHDA ELAPAYAVLK
210
SIIQAGCRVA IYLSERL
Length:217
Mass (Da):24,277
Last modified:November 2, 2010 - v3
Checksum:i37BEC3CEA621B77B
GO
Isoform 2 (identifier: Q99807-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-38: Missing.

Show »
Length:179
Mass (Da):20,158
Checksum:i9F9453EA670261DA
GO

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti45 – 451S → N in AAC69451 (PubMed:10501970).Curated
Sequence conflicti69 – 691G → W in CAB66582 (PubMed:11230166).Curated
Sequence conflicti72 – 721A → R in AAC69451 (PubMed:10501970).Curated
Sequence conflicti172 – 1721K → R in AAC69451 (PubMed:10501970).Curated

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti103 – 1031T → M.5 Publications
Corresponds to variant rs11074359 [ dbSNP | Ensembl ].
VAR_055148

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei1 – 3838Missing in isoform 2. 1 PublicationVSP_039068Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF098948 mRNA. Translation: AAD43648.1.
AL136647 mRNA. Translation: CAB66582.1.
AK024291 mRNA. Translation: BAB14876.1.
AK315470 mRNA. Translation: BAG37856.1.
AC099518 Genomic DNA. No translation available.
CH471186 Genomic DNA. Translation: EAW50268.1.
BC003185 mRNA. Translation: AAH03185.1.
AF032900 mRNA. Translation: AAC69451.1.
U81276 mRNA. Translation: AAC51120.1.
CCDSiCCDS10574.1. [Q99807-1]
CCDS53993.1. [Q99807-2]
RefSeqiNP_001177912.1. NM_001190983.1. [Q99807-2]
NP_057222.2. NM_016138.4. [Q99807-1]
UniGeneiHs.157113.

Genome annotation databases

EnsembliENST00000321998; ENSP00000322316; ENSG00000167186.
ENST00000544894; ENSP00000442923; ENSG00000167186. [Q99807-2]
ENST00000568985; ENSP00000456734; ENSG00000167186.
GeneIDi10229.
KEGGihsa:10229.
UCSCiuc002dfr.3. human. [Q99807-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF098948 mRNA. Translation: AAD43648.1.
AL136647 mRNA. Translation: CAB66582.1.
AK024291 mRNA. Translation: BAB14876.1.
AK315470 mRNA. Translation: BAG37856.1.
AC099518 Genomic DNA. No translation available.
CH471186 Genomic DNA. Translation: EAW50268.1.
BC003185 mRNA. Translation: AAH03185.1.
AF032900 mRNA. Translation: AAC69451.1.
U81276 mRNA. Translation: AAC51120.1.
CCDSiCCDS10574.1. [Q99807-1]
CCDS53993.1. [Q99807-2]
RefSeqiNP_001177912.1. NM_001190983.1. [Q99807-2]
NP_057222.2. NM_016138.4. [Q99807-1]
UniGeneiHs.157113.

3D structure databases

ProteinModelPortaliQ99807.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi9606.ENSP00000322316.

PTM databases

PhosphoSiteiQ99807.

Polymorphism and mutation databases

BioMutaiCOQ7.
DMDMi311033465.

Proteomic databases

MaxQBiQ99807.
PaxDbiQ99807.
PRIDEiQ99807.

Protocols and materials databases

DNASUi10229.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000321998; ENSP00000322316; ENSG00000167186.
ENST00000544894; ENSP00000442923; ENSG00000167186. [Q99807-2]
ENST00000568985; ENSP00000456734; ENSG00000167186.
GeneIDi10229.
KEGGihsa:10229.
UCSCiuc002dfr.3. human. [Q99807-1]

Organism-specific databases

CTDi10229.
GeneCardsiGC16P019078.
H-InvDBHIX0202253.
HGNCiHGNC:2244. COQ7.
HPAiHPA067252.
MIMi601683. gene.
neXtProtiNX_Q99807.
PharmGKBiPA26761.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiCOG2941.
GeneTreeiENSGT00390000014520.
HOGENOMiHOG000184972.
HOVERGENiHBG002214.
InParanoidiQ99807.
KOiK06134.
OMAiEGHEQVH.
OrthoDBiEOG73BVF0.
PhylomeDBiQ99807.
TreeFamiTF314559.

Enzyme and pathway databases

UniPathwayiUPA00232.
BioCyciMetaCyc:ENSG00000167186-MONOMER.
ReactomeiREACT_160111. Ubiquinol biosynthesis.

Miscellaneous databases

GeneWikiiCOQ7.
GenomeRNAii10229.
NextBioi38733.
PROiQ99807.
SOURCEiSearch...

Gene expression databases

BgeeiQ99807.
CleanExiHS_COQ7.
ExpressionAtlasiQ99807. baseline and differential.
GenevisibleiQ99807. HS.

Family and domain databases

Gene3Di1.20.1260.10. 1 hit.
HAMAPiMF_01658. COQ7.
InterProiIPR009078. Ferritin-like_SF.
IPR012347. Ferritin-rel.
IPR011566. Ubq_synth_Coq7.
[Graphical view]
PANTHERiPTHR11237. PTHR11237. 1 hit.
PfamiPF03232. COQ7. 1 hit.
[Graphical view]
SUPFAMiSSF47240. SSF47240. 1 hit.
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "Orthologues of the Caenorhabditis elegans longevity gene clk-1 in mouse and human."
    Asaumi S., Kuroyanagi H., Seki N., Shirasawa T.
    Genomics 58:293-301(1999) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), VARIANT MET-103.
  2. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2), VARIANT MET-103.
    Tissue: Brain.
  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] (ISOFORM 1), VARIANT MET-103.
  4. "The sequence and analysis of duplication-rich human chromosome 16."
    Martin J., Han C., Gordon L.A., Terry A., Prabhakar S., She X., Xie G., Hellsten U., Chan Y.M., Altherr M., Couronne O., Aerts A., Bajorek E., Black S., Blumer H., Branscomb E., Brown N.C., Bruno W.J.
    , Buckingham J.M., Callen D.F., Campbell C.S., Campbell M.L., Campbell E.W., Caoile C., Challacombe J.F., Chasteen L.A., Chertkov O., Chi H.C., Christensen M., Clark L.M., Cohn J.D., Denys M., Detter J.C., Dickson M., Dimitrijevic-Bussod M., Escobar J., Fawcett J.J., Flowers D., Fotopulos D., Glavina T., Gomez M., Gonzales E., Goodstein D., Goodwin L.A., Grady D.L., Grigoriev I., Groza M., Hammon N., Hawkins T., Haydu L., Hildebrand C.E., Huang W., Israni S., Jett J., Jewett P.B., Kadner K., Kimball H., Kobayashi A., Krawczyk M.-C., Leyba T., Longmire J.L., Lopez F., Lou Y., Lowry S., Ludeman T., Manohar C.F., Mark G.A., McMurray K.L., Meincke L.J., Morgan J., Moyzis R.K., Mundt M.O., Munk A.C., Nandkeshwar R.D., Pitluck S., Pollard M., Predki P., Parson-Quintana B., Ramirez L., Rash S., Retterer J., Ricke D.O., Robinson D.L., Rodriguez A., Salamov A., Saunders E.H., Scott D., Shough T., Stallings R.L., Stalvey M., Sutherland R.D., Tapia R., Tesmer J.G., Thayer N., Thompson L.S., Tice H., Torney D.C., Tran-Gyamfi M., Tsai M., Ulanovsky L.E., Ustaszewska A., Vo N., White P.S., Williams A.L., Wills P.L., Wu J.-R., Wu K., Yang J., DeJong P., Bruce D., Doggett N.A., Deaven L., Schmutz J., Grimwood J., Richardson P., Rokhsar D.S., Eichler E.E., Gilna P., Lucas S.M., Myers R.M., Rubin E.M., Pennacchio L.A.
    Nature 432:988-994(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  5. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  6. "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: Brain.
  7. "Conservation of the Caenorhabditis elegans timing gene clk-1 from yeast to human: a gene required for ubiquinone biosynthesis with potential implications for aging."
    Vajo Z., King L.M., Jonassen T., Wilkin D.J., Ho N., Munnich A., Clarke C.F., Francomano C.A.
    Mamm. Genome 10:1000-1004(1999) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 39-217 (ISOFORMS 1/2), VARIANT MET-103.
  8. "Structural and functional conservation of the Caenorhabditis elegans timing gene clk-1."
    Ewbank J.J., Barnes T.M., Lakowski B., Lussier M., Bussey H., Hekimi S.
    Science 275:980-983(1997) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 85-130 (ISOFORMS 1/2), VARIANT MET-103.
    Tissue: Placenta.
  9. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  10. Cited for: INTERACTION WITH ADCK4 AND COQ6.
  11. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
    Bian Y., Song C., Cheng K., Dong M., Wang F., Huang J., Sun D., Wang L., Ye M., Zou H.
    J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
    Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.
  12. Cited for: INTERACTION WITH COQ9.

Entry informationi

Entry nameiCOQ7_HUMAN
AccessioniPrimary (citable) accession number: Q99807
Secondary accession number(s): B2RDA9
, Q9BTT7, Q9H0T5, Q9UEW5, Q9UNR5
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: November 2, 2010
Last modified: July 22, 2015
This is version 142 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 16
    Human chromosome 16: 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

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.