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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

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

PharmGKBiPA143485439.

Polymorphism and mutation databases

BioMutaiCOQ10A.
DMDMi90111993.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 1515MitochondrionSequence analysisAdd
BLAST
Chaini16 – 247232Coenzyme Q-binding protein COQ10 homolog A, mitochondrialPRO_0000228644Add
BLAST

Proteomic databases

MaxQBiQ96MF6.
PaxDbiQ96MF6.
PRIDEiQ96MF6.

PTM databases

PhosphoSiteiQ96MF6.

Expressioni

Gene expression databases

BgeeiQ96MF6.
CleanExiHS_COQ10A.
ExpressionAtlasiQ96MF6. baseline and differential.
GenevisibleiQ96MF6. HS.

Interactioni

Subunit structurei

Interacts with coenzyme Q.By similarity

Protein-protein interaction databases

BioGridi124997. 1 interaction.
IntActiQ96MF6. 1 interaction.
MINTiMINT-6942683.
STRINGi9606.ENSP00000312587.

Structurei

3D structure databases

ProteinModelPortaliQ96MF6.
SMRiQ96MF6. Positions 91-230.
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.
OMAiLRFLMSC.
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 AAH26922.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated
The sequence AAQ89220.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti38 – 381L → P in BAB71339 (PubMed:14702039).Curated
Sequence conflicti202 – 2021H → R in BAB71339 (PubMed:14702039).Curated
Sequence conflicti213 – 2131V → A in BAB71339 (PubMed:14702039).Curated

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti79 – 791P → H.1 Publication
Corresponds to variant rs11543258 [ dbSNP | Ensembl ].
VAR_025703
Natural varianti231 – 2311P → S.
Corresponds to variant rs3184994 [ dbSNP | Ensembl ].
VAR_048828

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei1 – 4545MAWAG…PRPMR → MLLQVVREGKFSG in isoform 3. 1 PublicationVSP_046918Add
BLAST
Alternative sequencei1 – 4444MAWAG…PPRPM → MHSSSPSPTNDRGLRRTIRL APLSFGL in isoform 2. 1 PublicationVSP_017685Add
BLAST

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.
SMRiQ96MF6. Positions 91-230.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi124997. 1 interaction.
IntActiQ96MF6. 1 interaction.
MINTiMINT-6942683.
STRINGi9606.ENSP00000312587.

PTM databases

PhosphoSiteiQ96MF6.

Polymorphism and mutation databases

BioMutaiCOQ10A.
DMDMi90111993.

Proteomic databases

MaxQBiQ96MF6.
PaxDbiQ96MF6.
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.
neXtProtiNX_Q96MF6.
PharmGKBiPA143485439.
GenAtlasiSearch...

Phylogenomic databases

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

Miscellaneous databases

GenomeRNAii93058.
NextBioi77957.
PROiQ96MF6.

Gene expression databases

BgeeiQ96MF6.
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...

Publicationsi

« Hide 'large scale' publications
  1. "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] (ISOFORMS 1 AND 2).
    Tissue: Skeletal muscle.
  2. "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] (ISOFORMS 1 AND 3), VARIANT HIS-79.
    Tissue: Brain, Colon, Mammary gland and Skin.
  3. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 18-247 (ISOFORM 1).
  4. "The Saccharomyces cerevisiae COQ10 gene encodes a START domain protein required for function of coenzyme Q in respiration."
    Barros M.H., Johnson A., Gin P., Marbois B.N., Clarke C.F., Tzagoloff A.
    J. Biol. Chem. 280:42627-42635(2005) [PubMed] [Europe PMC] [Abstract]
    Cited for: PROBABLE FUNCTION.

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: March 16, 2016
This is version 101 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.