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Protein

Ubiquinone biosynthesis protein COQ9, mitochondrial

Gene

Coq9

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Lipid-binding protein involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration (PubMed:25339443). Binds a phospholipid of at least 10 carbons in each acyl group. May be required to present its bound-lipid to COQ7 (By similarity).By similarity2 Publications

Pathwayi: ubiquinone biosynthesis

This protein is involved in the pathway ubiquinone biosynthesis, which is part of Cofactor biosynthesis.2 Publications
View all proteins of this organism that are known to be involved in the pathway ubiquinone biosynthesis and in Cofactor biosynthesis.

GO - Molecular functioni

GO - Biological processi

  • mitochondrial electron transport, NADH to ubiquinone Source: MGI
  • ubiquinone biosynthetic process Source: UniProtKB

Keywordsi

Biological processUbiquinone biosynthesis
LigandLipid-binding

Enzyme and pathway databases

UniPathwayi
UPA00232

Names & Taxonomyi

Protein namesi
Recommended name:
Ubiquinone biosynthesis protein COQ9, mitochondrial
Gene namesi
Name:Coq9
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 8

Organism-specific databases

MGIiMGI:1915164 Coq9

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; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

Pathology & Biotechi

Disruption phenotypei

Encephalomyopathy due to a widespread coenzyme Q deficiency and accumulation of demethoxyubiquinone. Lethality between 3 and 6 months of age, due to neuronal death and demyelinization with severe vacuolization and astrogliosis in the brain.1 Publication

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 45MitochondrionSequence analysisAdd BLAST45
ChainiPRO_000022863846 – 313Ubiquinone biosynthesis protein COQ9, mitochondrialAdd BLAST268

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei81PhosphoserineCombined sources1
Modified residuei170N6-acetyllysineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiQ8K1Z0
MaxQBiQ8K1Z0
PaxDbiQ8K1Z0
PeptideAtlasiQ8K1Z0
PRIDEiQ8K1Z0

PTM databases

iPTMnetiQ8K1Z0
PhosphoSitePlusiQ8K1Z0

Expressioni

Gene expression databases

BgeeiENSMUSG00000031782 Expressed in 286 organ(s), highest expression level in heart
CleanExiMM_COQ9
ExpressionAtlasiQ8K1Z0 baseline and differential
GenevisibleiQ8K1Z0 MM

Interactioni

Subunit structurei

Homodimer. Interacts with COQ7.By similarity

GO - Molecular functioni

Protein-protein interaction databases

IntActiQ8K1Z0, 1 interactor
MINTiQ8K1Z0
STRINGi10090.ENSMUSP00000034234

Structurei

3D structure databases

ProteinModelPortaliQ8K1Z0
SMRiQ8K1Z0
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni236 – 239Lipid bindingBy similarity4

Domaini

Structurally similar to the bacterial FadR protein (fatty acid metabolism regulator protein).By similarity

Sequence similaritiesi

Belongs to the COQ9 family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG2969 Eukaryota
COG5590 LUCA
GeneTreeiENSGT00390000009328
HOGENOMiHOG000231991
HOVERGENiHBG079760
InParanoidiQ8K1Z0
KOiK18587
OMAiDEIWHAV
OrthoDBiEOG091G0M72
PhylomeDBiQ8K1Z0
TreeFamiTF324653

Family and domain databases

InterProiView protein in InterPro
IPR013718 COQ9
IPR012762 Ubiq_biosynth_COQ9
PANTHERiPTHR21427 PTHR21427, 1 hit
PfamiView protein in Pfam
PF08511 COQ9, 1 hit
TIGRFAMsiTIGR02396 diverge_rpsU, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

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

This entry has 1 described isoform and 2 potential isoforms that are computationally mapped.Show allAlign All

Q8K1Z0-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MAATAAVSGV LGRLGWRLLQ LRCLPVARCR PALVPRAFHT AVGFRSSEEQ
60 70 80 90 100
KQQPPHSSSQ QHSETQGPEF SRPPPRYTDQ SGEEEEDYES EEQLQHRILT
110 120 130 140 150
AALEFVPAHG WTAEAIAEGA QSLGLSSAAA SMFGSDGSEL ILHFVTQCNA
160 170 180 190 200
RLNQVLEEEQ KLVQLGQAEK RKTDQFLRDA VETRLRMLIP YIEHWPRALS
210 220 230 240 250
ILLLPHNIPP SLNLLTSMVD DMWHYAGDQS TDFNWYTRRA VLAGIYNTTE
260 270 280 290 300
LVMMQDSSPD FEDTWRFLEN RINDAMNMGH TAKQVKSTGE ALVQGLMGAA
310
VTLKNLTGLN QRR
Length:313
Mass (Da):35,083
Last modified:October 1, 2002 - v1
Checksum:i9B8D8FED0724F138
GO

Computationally mapped potential isoform sequencesi

There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
F6SFF5F6SFF5_MOUSE
Ubiquinone biosynthesis protein COQ...
Coq9
270Annotation score:
A0A1D5RLE9A0A1D5RLE9_MOUSE
Ubiquinone biosynthesis protein COQ...
Coq9
98Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti208I → N in BAB23347 (PubMed:16141072).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK004527 mRNA Translation: BAB23347.1
AK154343 mRNA Translation: BAE32528.1
AK158979 mRNA Translation: BAE34754.1
BC036386 mRNA Translation: AAH36386.1
CCDSiCCDS22550.1
RefSeqiNP_080728.1, NM_026452.2
UniGeneiMm.169234

Genome annotation databases

EnsembliENSMUST00000034234; ENSMUSP00000034234; ENSMUSG00000031782
GeneIDi67914
KEGGimmu:67914
UCSCiuc009mxd.1 mouse

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK004527 mRNA Translation: BAB23347.1
AK154343 mRNA Translation: BAE32528.1
AK158979 mRNA Translation: BAE34754.1
BC036386 mRNA Translation: AAH36386.1
CCDSiCCDS22550.1
RefSeqiNP_080728.1, NM_026452.2
UniGeneiMm.169234

3D structure databases

ProteinModelPortaliQ8K1Z0
SMRiQ8K1Z0
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiQ8K1Z0, 1 interactor
MINTiQ8K1Z0
STRINGi10090.ENSMUSP00000034234

PTM databases

iPTMnetiQ8K1Z0
PhosphoSitePlusiQ8K1Z0

Proteomic databases

EPDiQ8K1Z0
MaxQBiQ8K1Z0
PaxDbiQ8K1Z0
PeptideAtlasiQ8K1Z0
PRIDEiQ8K1Z0

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000034234; ENSMUSP00000034234; ENSMUSG00000031782
GeneIDi67914
KEGGimmu:67914
UCSCiuc009mxd.1 mouse

Organism-specific databases

CTDi57017
MGIiMGI:1915164 Coq9

Phylogenomic databases

eggNOGiKOG2969 Eukaryota
COG5590 LUCA
GeneTreeiENSGT00390000009328
HOGENOMiHOG000231991
HOVERGENiHBG079760
InParanoidiQ8K1Z0
KOiK18587
OMAiDEIWHAV
OrthoDBiEOG091G0M72
PhylomeDBiQ8K1Z0
TreeFamiTF324653

Enzyme and pathway databases

UniPathwayi
UPA00232

Miscellaneous databases

PROiPR:Q8K1Z0
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000031782 Expressed in 286 organ(s), highest expression level in heart
CleanExiMM_COQ9
ExpressionAtlasiQ8K1Z0 baseline and differential
GenevisibleiQ8K1Z0 MM

Family and domain databases

InterProiView protein in InterPro
IPR013718 COQ9
IPR012762 Ubiq_biosynth_COQ9
PANTHERiPTHR21427 PTHR21427, 1 hit
PfamiView protein in Pfam
PF08511 COQ9, 1 hit
TIGRFAMsiTIGR02396 diverge_rpsU, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiCOQ9_MOUSE
AccessioniPrimary (citable) accession number: Q8K1Z0
Secondary accession number(s): Q9CT61
Entry historyiIntegrated into UniProtKB/Swiss-Prot: March 21, 2006
Last sequence update: October 1, 2002
Last modified: September 12, 2018
This is version 118 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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