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Entry version 174 (08 May 2019)
Sequence version 3 (02 Nov 2010)
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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

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the ‘protein existence’ evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>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

<p>This subsection of the ‘Function’ section provides information relevant to cofactors. A cofactor is any non-protein substance required for a protein to be catalytically active. Some cofactors are inorganic, such as the metal atoms zinc, iron, and copper in various oxidation states. Others, such as most vitamins, are organic.<p><a href='/help/cofactor' target='_top'>More...</a></p>Cofactori

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

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section describes the metabolic pathway(s) associated with a protein.<p><a href='/help/pathway' target='_top'>More...</a></p>Pathwayi: ubiquinone 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)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Function’ section indicates at which position the protein binds a given metal ion. The nature of the metal is indicated in the ‘Description’ field.<p><a href='/help/metal' target='_top'>More...</a></p>Metal bindingi60Iron 1UniRule annotation1
Metal bindingi90Iron 1UniRule annotation1
Metal bindingi90Iron 2UniRule annotation1
Metal bindingi93Iron 1UniRule annotation1
Metal bindingi142Iron 2UniRule annotation1
Metal bindingi178Iron 1UniRule annotation1
Metal bindingi178Iron 2UniRule annotation1
Metal bindingi181Iron 2UniRule annotation1

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Molecular functionMonooxygenase, Oxidoreductase
Biological processUbiquinone biosynthesis
LigandIron, Metal-binding

Enzyme and pathway databases

BioCyc Collection of Pathway/Genome Databases

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BioCyci
MetaCyc:ENSG00000167186-MONOMER

Reactome - a knowledgebase of biological pathways and processes

More...
Reactomei
R-HSA-2142789 Ubiquinol biosynthesis

UniPathway: a resource for the exploration and annotation of metabolic pathways

More...
UniPathwayi
UPA00232

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>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
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: ‘Name’, ‘Synonyms’, ‘Ordered locus names’ and ‘ORF names’.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:COQ7UniRule annotation
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the ‘taxonomic identifier’ or ‘taxid’.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes_manual">proteome</a> can consist of several components. <br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 16

Organism-specific databases

Human Gene Nomenclature Database

More...
HGNCi
HGNC:2244 COQ7

Online Mendelian Inheritance in Man (OMIM)

More...
MIMi
601683 gene

neXtProt; the human protein knowledge platform

More...
neXtProti
NX_Q99807

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte & Seán O’Donoghue; Source: COMPARTMENTS

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the ‘Pathology and Biotech’ section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

Coenzyme Q10 deficiency, primary, 8 (COQ10D8)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionAn autosomal recessive disorder resulting from mitochondrial dysfunction and characterized by decreased levels of coenzyme Q10. Patients manifest neonatal lung hypoplasia, contractures, early infantile hypertension and cardiac hypertrophy, secondary to prenatal kidney dysplasia, with neonatal and infantile renal dysfunction. Clinical features also include progressive peripheral neuropathy, muscular hypotonia and atrophy, and mild psychomotor delay with hearing and visual impairment.
See also OMIM:616733
Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_076370141V → E in COQ10D8. 1 PublicationCorresponds to variant dbSNP:rs864321686EnsemblClinVar.1

Keywords - Diseasei

Disease mutation, Primary mitochondrial disease

Organism-specific databases

DisGeNET

More...
DisGeNETi
10229

GeneReviews a resource of expert-authored, peer-reviewed disease descriptions.

More...
GeneReviewsi
COQ7

MalaCards human disease database

More...
MalaCardsi
COQ7
MIMi616733 phenotype

Open Targets

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OpenTargetsi
ENSG00000167186

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...
PharmGKBi
PA26761

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

More...
BioMutai
COQ7

Domain mapping of disease mutations (DMDM)

More...
DMDMi
311033465

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a transit peptide.<p><a href='/help/transit' target='_top'>More...</a></p>Transit peptidei1 – 35MitochondrionUniRule annotationAdd BLAST35
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_000007925136 – 2175-demethoxyubiquinone hydroxylase, mitochondrialAdd BLAST182

Proteomic databases

Encyclopedia of Proteome Dynamics

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EPDi
Q99807

jPOST - Japan Proteome Standard Repository/Database

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jPOSTi
Q99807

MaxQB - The MaxQuant DataBase

More...
MaxQBi
Q99807

PaxDb, a database of protein abundance averages across all three domains of life

More...
PaxDbi
Q99807

PeptideAtlas

More...
PeptideAtlasi
Q99807

PRoteomics IDEntifications database

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PRIDEi
Q99807

ProteomicsDB human proteome resource

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ProteomicsDBi
78485
78486 [Q99807-2]

Consortium for Top Down Proteomics

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TopDownProteomicsi
Q99807-1 [Q99807-1]
Q99807-2 [Q99807-2]

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

More...
iPTMneti
Q99807

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

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PhosphoSitePlusi
Q99807

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

<p>This subsection of the ‘Expression’ section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms. By default, the information is derived from experiments at the mRNA level, unless specified ‘at protein level’. <br></br>Examples: <a href="http://www.uniprot.org/uniprot/P92958#expression">P92958</a>, <a href="http://www.uniprot.org/uniprot/Q8TDN4#expression">Q8TDN4</a>, <a href="http://www.uniprot.org/uniprot/O14734#expression">O14734</a><p><a href='/help/tissue_specificity' target='_top'>More...</a></p>Tissue specificityi

Expressed dominantly in heart and skeletal muscle.

Gene expression databases

Bgee dataBase for Gene Expression Evolution

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Bgeei
ENSG00000167186 Expressed in 179 organ(s), highest expression level in testis

ExpressionAtlas, Differential and Baseline Expression

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ExpressionAtlasi
Q99807 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

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Genevisiblei
Q99807 HS

Organism-specific databases

Human Protein Atlas

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HPAi
HPA067252
HPA071922

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction_section">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>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 COQ8B and COQ6 (PubMed:24270420). Interacts with COQ9 (PubMed:25339443).UniRule annotation2 Publications

<p>This subsection of the '<a href="http://www.uniprot.org/help/interaction_section%27">Interaction</a> section provides information about binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="http://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated on a monthly basis. Each binary interaction is displayed on a separate line.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

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BioGridi
115523, 24 interactors

ComplexPortal: manually curated resource of macromolecular complexes

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ComplexPortali
CPX-3642 CoQ biosynthetic complex

Database of interacting proteins

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DIPi
DIP-62092N

Protein interaction database and analysis system

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IntActi
Q99807, 29 interactors

STRING: functional protein association networks

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STRINGi
9606.ENSP00000322316

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

3D structure databases

SWISS-MODEL Repository - a database of annotated 3D protein structure models

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SMRi
Q99807

Database of comparative protein structure models

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ModBasei
Search...

MobiDB: a database of protein disorder and mobility annotations

More...
MobiDBi
Search...

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Family and Domains’ section indicates the positions and types of repeated sequence motifs or repeated domains within the protein.<p><a href='/help/repeat' target='_top'>More...</a></p>Repeati48 – 1291Add BLAST82
Repeati130 – 2172Add BLAST88

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Family and Domains’ section describes a region of interest that cannot be described in other subsections.<p><a href='/help/region' target='_top'>More...</a></p>Regioni48 – 2172 X approximate tandem repeatsAdd BLAST170

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Family and Domains’ section describes the position of regions of compositional bias within the protein and the particular amino acids that are over-represented within those regions.<p><a href='/help/compbias' target='_top'>More...</a></p>Compositional biasi4 – 9Poly-Ala6

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Belongs to the COQ7 family.UniRule annotation

Keywords - Domaini

Repeat, Transit peptide

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

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eggNOGi
KOG4061 Eukaryota
COG2941 LUCA

Ensembl GeneTree

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GeneTreei
ENSGT00390000014520

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

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HOGENOMi
HOG000184972

InParanoid: Eukaryotic Ortholog Groups

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InParanoidi
Q99807

KEGG Orthology (KO)

More...
KOi
K06134

Identification of Orthologs from Complete Genome Data

More...
OMAi
TIGHMWE

Database of Orthologous Groups

More...
OrthoDBi
1242061at2759

Database for complete collections of gene phylogenies

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PhylomeDBi
Q99807

TreeFam database of animal gene trees

More...
TreeFami
TF314559

Family and domain databases

Conserved Domains Database

More...
CDDi
cd01042 DMQH, 1 hit

HAMAP database of protein families

More...
HAMAPi
MF_01658 COQ7, 1 hit

Integrated resource of protein families, domains and functional sites

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InterProi
View protein in InterPro
IPR009078 Ferritin-like_SF
IPR011566 Ubq_synth_Coq7

The PANTHER Classification System

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PANTHERi
PTHR11237 PTHR11237, 1 hit

Pfam protein domain database

More...
Pfami
View protein in Pfam
PF03232 COQ7, 1 hit

Superfamily database of structural and functional annotation

More...
SUPFAMi
SSF47240 SSF47240, 1 hit

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>.<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (2+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 <p>This subsection of the ‘Sequence’ section lists the alternative protein sequences (isoforms) that can be generated from the same gene by a single or by the combination of up to four biological events (alternative promoter usage, alternative splicing, alternative initiation and ribosomal frameshifting). Additionally, this section gives relevant information on each alternative protein isoform.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 2 described isoforms and 5 potential isoforms that are computationally mapped.Show allAlign All

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
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> 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

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There are 5 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
H3BP28H3BP28_HUMAN
5-demethoxyubiquinone hydroxylase, ...
COQ7
194Annotation score:

Annotation score:3 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
H3BTN8H3BTN8_HUMAN
5-demethoxyubiquinone hydroxylase, ...
COQ7
130Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
H3BSZ3H3BSZ3_HUMAN
5-demethoxyubiquinone hydroxylase, ...
COQ7
103Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
I3L1T0I3L1T0_HUMAN
5-demethoxyubiquinone hydroxylase, ...
COQ7 hCG_1743024
136Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
H3BS11H3BS11_HUMAN
5-demethoxyubiquinone hydroxylase, ...
COQ7
106Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti45S → N in AAC69451 (PubMed:10501970).Curated1
Sequence conflicti69G → W in CAB66582 (PubMed:11230166).Curated1
Sequence conflicti72A → R in AAC69451 (PubMed:10501970).Curated1
Sequence conflicti172K → R in AAC69451 (PubMed:10501970).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_055148103T → M5 PublicationsCorresponds to variant dbSNP:rs11074359Ensembl.1
Natural variantiVAR_076370141V → E in COQ10D8. 1 PublicationCorresponds to variant dbSNP:rs864321686EnsemblClinVar.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes the sequence of naturally occurring alternative protein isoform(s). The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_0390681 – 38Missing in isoform 2. 1 PublicationAdd BLAST38

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

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EMBLi

GenBank nucleotide sequence database

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GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

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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

The Consensus CDS (CCDS) project

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CCDSi
CCDS10574.1 [Q99807-1]
CCDS53993.1 [Q99807-2]

NCBI Reference Sequences

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RefSeqi
NP_001177912.1, NM_001190983.1 [Q99807-2]
NP_057222.2, NM_016138.4 [Q99807-1]

Genome annotation databases

Ensembl eukaryotic genome annotation project

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Ensembli
ENST00000321998; ENSP00000322316; ENSG00000167186 [Q99807-1]
ENST00000544894; ENSP00000442923; ENSG00000167186 [Q99807-2]
ENST00000568985; ENSP00000456734; ENSG00000167186 [Q99807-1]

Database of genes from NCBI RefSeq genomes

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GeneIDi
10229

KEGG: Kyoto Encyclopedia of Genes and Genomes

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KEGGi
hsa:10229

UCSC genome browser

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UCSCi
uc002dfr.4 human [Q99807-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>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]

3D structure databases

SMRiQ99807
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi115523, 24 interactors
ComplexPortaliCPX-3642 CoQ biosynthetic complex
DIPiDIP-62092N
IntActiQ99807, 29 interactors
STRINGi9606.ENSP00000322316

PTM databases

iPTMnetiQ99807
PhosphoSitePlusiQ99807

Polymorphism and mutation databases

BioMutaiCOQ7
DMDMi311033465

Proteomic databases

EPDiQ99807
jPOSTiQ99807
MaxQBiQ99807
PaxDbiQ99807
PeptideAtlasiQ99807
PRIDEiQ99807
ProteomicsDBi78485
78486 [Q99807-2]
TopDownProteomicsiQ99807-1 [Q99807-1]
Q99807-2 [Q99807-2]

Protocols and materials databases

The DNASU plasmid repository

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DNASUi
10229
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000321998; ENSP00000322316; ENSG00000167186 [Q99807-1]
ENST00000544894; ENSP00000442923; ENSG00000167186 [Q99807-2]
ENST00000568985; ENSP00000456734; ENSG00000167186 [Q99807-1]
GeneIDi10229
KEGGihsa:10229
UCSCiuc002dfr.4 human [Q99807-1]

Organism-specific databases

Comparative Toxicogenomics Database

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CTDi
10229
DisGeNETi10229

GeneCards: human genes, protein and diseases

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GeneCardsi
COQ7
GeneReviewsiCOQ7

H-Invitational Database, human transcriptome db

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H-InvDBi
HIX0202253
HGNCiHGNC:2244 COQ7
HPAiHPA067252
HPA071922
MalaCardsiCOQ7
MIMi601683 gene
616733 phenotype
neXtProtiNX_Q99807
OpenTargetsiENSG00000167186
PharmGKBiPA26761

GenAtlas: human gene database

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GenAtlasi
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Phylogenomic databases

eggNOGiKOG4061 Eukaryota
COG2941 LUCA
GeneTreeiENSGT00390000014520
HOGENOMiHOG000184972
InParanoidiQ99807
KOiK06134
OMAiTIGHMWE
OrthoDBi1242061at2759
PhylomeDBiQ99807
TreeFamiTF314559

Enzyme and pathway databases

UniPathwayi
UPA00232

BioCyciMetaCyc:ENSG00000167186-MONOMER
ReactomeiR-HSA-2142789 Ubiquinol biosynthesis

Miscellaneous databases

The Gene Wiki collection of pages on human genes and proteins

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GeneWikii
COQ7

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

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GenomeRNAii
10229

Protein Ontology

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PROi
PR:Q99807

The Stanford Online Universal Resource for Clones and ESTs

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SOURCEi
Search...

Gene expression databases

BgeeiENSG00000167186 Expressed in 179 organ(s), highest expression level in testis
ExpressionAtlasiQ99807 baseline and differential
GenevisibleiQ99807 HS

Family and domain databases

CDDicd01042 DMQH, 1 hit
HAMAPiMF_01658 COQ7, 1 hit
InterProiView protein in InterPro
IPR009078 Ferritin-like_SF
IPR011566 Ubq_synth_Coq7
PANTHERiPTHR11237 PTHR11237, 1 hit
PfamiView protein in Pfam
PF03232 COQ7, 1 hit
SUPFAMiSSF47240 SSF47240, 1 hit

ProtoNet; Automatic hierarchical classification of proteins

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ProtoNeti
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<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the ‘Entry information’ section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiCOQ7_HUMAN
<p>This subsection of the ‘Entry information’ section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called ‘Primary (citable) accession number’.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: Q99807
Secondary accession number(s): B2RDA9
, Q9BTT7, Q9H0T5, Q9UEW5, Q9UNR5
<p>This subsection of the ‘Entry information’ section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification (‘Last modified’). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: November 2, 2010
Last modified: May 8, 2019
This is version 174 of the entry and version 3 of the sequence. See complete history.
<p>This subsection of the ‘Entry information’ section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>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.

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>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. SIMILARITY comments
    Index of protein domains and families
  3. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  6. PATHWAY comments
    Index of metabolic and biosynthesis pathways
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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