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Protein

Coenzyme Q-binding protein COQ10 homolog A, mitochondrial

Gene

COQ10A

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 3 out of 5-Experimental evidence at transcript leveli

Functioni

Required for the function of coenzyme Q in the respiratory chain. May serve as a chaperone or may be involved in the transport of Q6 from its site of synthesis to the catalytic sites of the respiratory complexes (Probable).Curated

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Enzyme and pathway databases

BioCyciZFISH:ENSG00000135469-MONOMER.
ReactomeiR-HSA-611105. Respiratory electron transport.

Names & Taxonomyi

Protein namesi
Recommended name:
Coenzyme Q-binding protein COQ10 homolog A, mitochondrial
Gene namesi
Name:COQ10A
ORF Names:UNQ6192/PRO20219
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 12

Organism-specific databases

HGNCiHGNC:26515. COQ10A.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

OpenTargetsiENSG00000135469.
PharmGKBiPA143485439.

Polymorphism and mutation databases

BioMutaiCOQ10A.
DMDMi90111993.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 15MitochondrionSequence analysisAdd BLAST15
ChainiPRO_000022864416 – 247Coenzyme Q-binding protein COQ10 homolog A, mitochondrialAdd BLAST232

Proteomic databases

MaxQBiQ96MF6.
PaxDbiQ96MF6.
PeptideAtlasiQ96MF6.
PRIDEiQ96MF6.

PTM databases

iPTMnetiQ96MF6.
PhosphoSitePlusiQ96MF6.

Expressioni

Gene expression databases

BgeeiENSG00000135469.
CleanExiHS_COQ10A.
ExpressionAtlasiQ96MF6. baseline and differential.
GenevisibleiQ96MF6. HS.

Organism-specific databases

HPAiHPA068931.

Interactioni

Subunit structurei

Interacts with coenzyme Q.By similarity

Protein-protein interaction databases

BioGridi124997. 4 interactors.
IntActiQ96MF6. 1 interactor.
MINTiMINT-6942683.
STRINGi9606.ENSP00000312587.

Structurei

3D structure databases

ProteinModelPortaliQ96MF6.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the COQ10 family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG3177. Eukaryota.
COG2867. LUCA.
GeneTreeiENSGT00390000006193.
HOGENOMiHOG000261732.
HOVERGENiHBG081329.
InParanoidiQ96MF6.
KOiK18588.
OMAiFEVVSNV.
OrthoDBiEOG091G0POH.
PhylomeDBiQ96MF6.
TreeFamiTF314447.

Family and domain databases

Gene3Di3.30.530.20. 1 hit.
InterProiIPR005031. COQ10_START.
IPR023393. START-like_dom.
[Graphical view]
PfamiPF03364. Polyketide_cyc. 1 hit.
[Graphical view]

Sequences (3)i

Sequence statusi: Complete.

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

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q96MF6-1) [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
MAWAGSRRVP AGTRAAAERC CRLSLSPGAQ PAPPPGPLPP PRPMRFLTSC
60 70 80 90 100
SLLLPRAAQI LAAEAGLPSS RSFMGFAAPF TNKRKAYSER RIMGYSMQEM
110 120 130 140 150
YEVVSNVQEY REFVPWCKKS LVVSSRKGHL KAQLEVGFPP VMERYTSAVS
160 170 180 190 200
MVKPHMVKAV CTDGKLFNHL ETIWRFSPGI PAYPRTCTVD FSISFEFRSL
210 220 230 240
LHSQLATMFF DEVVKQNVAA FERRAATKFG PETAIPRELM FHEVHQT
Length:247
Mass (Da):27,686
Last modified:March 21, 2006 - v2
Checksum:i148299DE0704A8B8
GO
Isoform 2 (identifier: Q96MF6-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-44: MAWAGSRRVPAGTRAAAERCCRLSLSPGAQPAPPPGPLPPPRPM → MHSSSPSPTNDRGLRRTIRLAPLSFGL

Note: No experimental confirmation available.
Show »
Length:230
Mass (Da):26,120
Checksum:iAD69752664DF5F99
GO
Isoform 3 (identifier: Q96MF6-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-45: MAWAGSRRVPAGTRAAAERCCRLSLSPGAQPAPPPGPLPPPRPMR → MLLQVVREGKFSG

Note: No experimental confirmation available.
Show »
Length:215
Mass (Da):24,460
Checksum:i442DECCE308C07FA
GO

Sequence cautioni

The sequence AAH26922 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated
The sequence AAQ89220 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti38L → P in BAB71339 (PubMed:14702039).Curated1
Sequence conflicti202H → R in BAB71339 (PubMed:14702039).Curated1
Sequence conflicti213V → A in BAB71339 (PubMed:14702039).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_02570379P → H.1 PublicationCorresponds to variant rs11543258dbSNPEnsembl.1
Natural variantiVAR_048828231P → S.Corresponds to variant rs3184994dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0469181 – 45MAWAG…PRPMR → MLLQVVREGKFSG in isoform 3. 1 PublicationAdd BLAST45
Alternative sequenceiVSP_0176851 – 44MAWAG…PPRPM → MHSSSPSPTNDRGLRRTIRL APLSFGL in isoform 2. 1 PublicationAdd BLAST44

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK057003 mRNA. Translation: BAB71339.1.
AK057014 mRNA. Translation: BAB71344.1.
BC002435 mRNA. Translation: AAH02435.2.
BC026922 mRNA. Translation: AAH26922.1. Different initiation.
BC047444 mRNA. Translation: AAH47444.1.
BC073923 mRNA. Translation: AAH73923.1.
AY358861 mRNA. Translation: AAQ89220.1. Different initiation.
CCDSiCCDS41796.1. [Q96MF6-1]
CCDS44921.1. [Q96MF6-3]
RefSeqiNP_001092807.1. NM_001099337.1. [Q96MF6-3]
NP_653177.3. NM_144576.3. [Q96MF6-1]
UniGeneiHs.4096.

Genome annotation databases

EnsembliENST00000308197; ENSP00000312587; ENSG00000135469. [Q96MF6-1]
ENST00000433805; ENSP00000407843; ENSG00000135469. [Q96MF6-3]
ENST00000546544; ENSP00000446723; ENSG00000135469. [Q96MF6-2]
GeneIDi93058.
KEGGihsa:93058.
UCSCiuc001sko.5. human. [Q96MF6-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK057003 mRNA. Translation: BAB71339.1.
AK057014 mRNA. Translation: BAB71344.1.
BC002435 mRNA. Translation: AAH02435.2.
BC026922 mRNA. Translation: AAH26922.1. Different initiation.
BC047444 mRNA. Translation: AAH47444.1.
BC073923 mRNA. Translation: AAH73923.1.
AY358861 mRNA. Translation: AAQ89220.1. Different initiation.
CCDSiCCDS41796.1. [Q96MF6-1]
CCDS44921.1. [Q96MF6-3]
RefSeqiNP_001092807.1. NM_001099337.1. [Q96MF6-3]
NP_653177.3. NM_144576.3. [Q96MF6-1]
UniGeneiHs.4096.

3D structure databases

ProteinModelPortaliQ96MF6.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi124997. 4 interactors.
IntActiQ96MF6. 1 interactor.
MINTiMINT-6942683.
STRINGi9606.ENSP00000312587.

PTM databases

iPTMnetiQ96MF6.
PhosphoSitePlusiQ96MF6.

Polymorphism and mutation databases

BioMutaiCOQ10A.
DMDMi90111993.

Proteomic databases

MaxQBiQ96MF6.
PaxDbiQ96MF6.
PeptideAtlasiQ96MF6.
PRIDEiQ96MF6.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000308197; ENSP00000312587; ENSG00000135469. [Q96MF6-1]
ENST00000433805; ENSP00000407843; ENSG00000135469. [Q96MF6-3]
ENST00000546544; ENSP00000446723; ENSG00000135469. [Q96MF6-2]
GeneIDi93058.
KEGGihsa:93058.
UCSCiuc001sko.5. human. [Q96MF6-1]

Organism-specific databases

CTDi93058.
GeneCardsiCOQ10A.
HGNCiHGNC:26515. COQ10A.
HPAiHPA068931.
neXtProtiNX_Q96MF6.
OpenTargetsiENSG00000135469.
PharmGKBiPA143485439.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG3177. Eukaryota.
COG2867. LUCA.
GeneTreeiENSGT00390000006193.
HOGENOMiHOG000261732.
HOVERGENiHBG081329.
InParanoidiQ96MF6.
KOiK18588.
OMAiFEVVSNV.
OrthoDBiEOG091G0POH.
PhylomeDBiQ96MF6.
TreeFamiTF314447.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000135469-MONOMER.
ReactomeiR-HSA-611105. Respiratory electron transport.

Miscellaneous databases

GenomeRNAii93058.
PROiQ96MF6.

Gene expression databases

BgeeiENSG00000135469.
CleanExiHS_COQ10A.
ExpressionAtlasiQ96MF6. baseline and differential.
GenevisibleiQ96MF6. HS.

Family and domain databases

Gene3Di3.30.530.20. 1 hit.
InterProiIPR005031. COQ10_START.
IPR023393. START-like_dom.
[Graphical view]
PfamiPF03364. Polyketide_cyc. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiCQ10A_HUMAN
AccessioniPrimary (citable) accession number: Q96MF6
Secondary accession number(s): Q6GMR6
, Q6UWB9, Q86X16, Q8TAL2, Q96MF1, Q9BUP4
Entry historyi
Integrated into UniProtKB/Swiss-Prot: March 21, 2006
Last sequence update: March 21, 2006
Last modified: November 30, 2016
This is version 107 of the entry and version 2 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 12
    Human chromosome 12: 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. SIMILARITY comments
    Index of protein domains and families

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 one 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.