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Protein

Coenzyme Q-binding protein COQ10 homolog A, mitochondrial

Gene

COQ10A

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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

  • ubiquinone binding Source: GO_Central

GO - Biological processi

Enzyme and pathway databases

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

EuPathDBiHostDB:ENSG00000135469.13
HGNCiHGNC:26515 COQ10A
neXtProtiNX_Q96MF6

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

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi93058
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
ProteomicsDBi77348
77349 [Q96MF6-2]

PTM databases

iPTMnetiQ96MF6
PhosphoSitePlusiQ96MF6

Expressioni

Gene expression databases

BgeeiENSG00000135469 Expressed in 187 organ(s), highest expression level in deltoid
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, 3 interactors
IntActiQ96MF6, 62 interactors
MINTiQ96MF6
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
OMAiAAQRCCG
OrthoDBiEOG091G0POH
PhylomeDBiQ96MF6
TreeFamiTF314447

Family and domain databases

Gene3Di3.30.530.20, 1 hit
InterProiView protein in InterPro
IPR005031 COQ10_START
IPR023393 START-like_dom_sf
PfamiView protein in Pfam
PF03364 Polyketide_cyc, 1 hit

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

This entry has 3 described isoforms and 2 potential isoforms that are computationally mapped.Show allAlign All

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

Computationally mapped potential isoform sequencesi

There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
H0YHF1H0YHF1_HUMAN
Coenzyme Q-binding protein COQ10 ho...
COQ10A
153Annotation score:
H0YHW3H0YHW3_HUMAN
Coenzyme Q-binding protein COQ10 ho...
COQ10A
64Annotation score:

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 → H1 PublicationCorresponds to variant dbSNP:rs11543258Ensembl.1
Natural variantiVAR_048828231P → S. Corresponds to variant dbSNP:rs3184994Ensembl.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

Similar proteinsi

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, 3 interactors
IntActiQ96MF6, 62 interactors
MINTiQ96MF6
STRINGi9606.ENSP00000312587

PTM databases

iPTMnetiQ96MF6
PhosphoSitePlusiQ96MF6

Polymorphism and mutation databases

BioMutaiCOQ10A
DMDMi90111993

Proteomic databases

MaxQBiQ96MF6
PaxDbiQ96MF6
PeptideAtlasiQ96MF6
PRIDEiQ96MF6
ProteomicsDBi77348
77349 [Q96MF6-2]

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
DisGeNETi93058
EuPathDBiHostDB:ENSG00000135469.13
GeneCardsiCOQ10A
HGNCiHGNC:26515 COQ10A
HPAiHPA068931
neXtProtiNX_Q96MF6
OpenTargetsiENSG00000135469
PharmGKBiPA143485439
GenAtlasiSearch...

Phylogenomic databases

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

Enzyme and pathway databases

ReactomeiR-HSA-611105 Respiratory electron transport

Miscellaneous databases

ChiTaRSiCOQ10A human
GenomeRNAii93058
PROiPR:Q96MF6

Gene expression databases

BgeeiENSG00000135469 Expressed in 187 organ(s), highest expression level in deltoid
CleanExiHS_COQ10A
ExpressionAtlasiQ96MF6 baseline and differential
GenevisibleiQ96MF6 HS

Family and domain databases

Gene3Di3.30.530.20, 1 hit
InterProiView protein in InterPro
IPR005031 COQ10_START
IPR023393 START-like_dom_sf
PfamiView protein in Pfam
PF03364 Polyketide_cyc, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiCQ10A_HUMAN
AccessioniPrimary (citable) accession number: Q96MF6
Secondary accession number(s): Q6GMR6
, Q6UWB9, Q86X16, Q8TAL2, Q96MF1, Q9BUP4
Entry historyiIntegrated into UniProtKB/Swiss-Prot: March 21, 2006
Last sequence update: March 21, 2006
Last modified: October 10, 2018
This is version 118 of the entry and version 2 of the sequence. See 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. SIMILARITY comments
    Index of protein domains and families
  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. Human chromosome 12
    Human chromosome 12: entries, gene names and cross-references to MIM
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Main funding by: National Institutes of Health

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